**Management for the N0 Neck of SCC in the Oral Cavity**

Masaya Okura, Natsuko Yoshimura Sawai, Satoshi Sumioka and Tomonao Aikawa *The First Department of Oral and Maxillofacial Surgery, Osaka University Graduate School of Dentistry, Japan* 

#### **1. Introduction**

112 Neck Dissection – Clinical Application and Recent Advances

Medina JE, Lorè JM. (2005) The neck, In: Lorè JM, Medina JE, *Anatomic atlas of head and neck* 

Salgarelli AC, Landini B, Bellini P, Multinu A, Consolo U, Collini M. (2009) A simple

Soo KC, Guiloff RJ, Oh A, Della Rovere GQ, Westbury G (1986) Innervation of the trapezius muscle: a study in patients undergoing neck dissection. *Head Neck Surg* 12: 488–95. Suarez O (1963) El problema de las metastasis linfaticas y alejadas del cacer de laringe e

Symes A, Ellis H (2005) Variations in the surface anatomy of the spinal accessory nerve in

Younossi-Hartenstein A, Jones M, Hartenstein V (2001) Embryonic development of the

nervous system of the temnocephalid flatworm Craspedella pedum. *J Comp Neurol*

method of identifying the spinal accessory nerve in modified radical neck dissection: anatomic study and clinical implications for resident training. *Oral* 

*surgery*, 804, Elsevier, Philadelphia.

hipofargine. *Rev Otorhinolaryngol* 23: 83–9.

the posterior triangle. *Surg Radiol Anat* 27: 404–8.

*Maxillofac Surg* 13: 69-72.

435: 259–62.

The oral cavity is the most predominant location in the head and neck region for primary malignant tumors, and more than 90 % cancer consists of squamous cell carcinoma (SCC).(Shah and Patel 2003) SCC has a high propensity to early and extensive lymph node metastases. Regarding cancer stage distribution at diagnosis, regional spread is more frequent in cancers of oral cavity and pharynx compared to other cancers, including such as prostate, breast, lung and bronchus, and colorectum (Figure 1).(Jemal *et al.* 2010) Therefore, clinicians for cancers of the oral cavity and pharynx have to regard regional metastasis as most important. Advanced SCC of the oral cavity has regional metastasis frequently, and even in small tumors (T1 or T2) has a relatively high propensity of regional lymph node

Fig. 1. Regional stage distribution of selected cancers, United States, 1999 to 2005. Source: Horner M, Ries L, Krapcho M, et al, eds. SEER Cancer Statistics Review, 1975-2006. Bethesda, MD: National Cancer Institute; 2009.

Management for the N0 Neck of SCC in the Oral Cavity 115

 - + - + - + Pathologically negative1 65 15 27 5 39 3

Therapeutic neck dissection 2 24 1 16 1 12 Elective neck dissection 5 0 2 0 1 0 Regional recurrence 16 1 7 0 6 2 Loco-regional recurrence 4 0 2 0 2 0 Total 92 40 39 21 49 17 Sensitivity 52% 62% 67% Specificity 81% 84% 93% Positive predictive value 63% 76% 82% Negative predictive value 74% 73% 83% Accuracy 70% 74% 83% Table 1. Image accuracy of nodal positivity in patients with SCC of the oral tongue. 1Pathologically negative includes patients who performed intraoral excision alone with no regional recurrence; CT, computed tomography; MRI, magnetic resonance imaging; US, ultrasound sonography; -, negative; +, positive. Number indicates patient number.

The N0 neck can be treated electively or can be carefully observed (wait-and-see), and the decision can be made from each own clinical experience (Table 1). Randomized controlled trial (RCT) is desired to determine which is preferred, however RCT is not easy task. So far four RCTs of the small sample size had been performed. Vandenbrouck et al.(Vandenbrouck *et al.* 1980) demonstrated that the survival rates were similar between two treatment arms in 75 patients with oral cavity cancer, whereas Fakih et al.(Fakih *et al.* 1989) (n = 70) and Klingerman et al.(Kligerman *et al.* 1994) (n = 67) found that elective neck dissection had significant benefit for patients with tumor thickness of more than 4 mm. In 2009, Yuen et al.(Yuen *et al.* 2009) demonstrated that disease-free survival was quite similar between two arms in 71 patients with SCC of the oral tongue. Thus, these four RCTs failed to impact on clinicians due to the inconsistency and small number of cases studied. In 1994 Weiss et al.(Weiss *et al.* 1994) created a decision tree analysis and demonstrated that when the probability of occult cervical metastasis is more than 20%, the neck should be electively treated. Since then a large number of studies(Dias *et al.* 2001; Ferlito *et al.* 2006; Greenberg *et al.* 2003; Haddadin *et al.* 1999; O'brien *et al.* 2000; Sano and Myers 2007; Wei *et al.* 2006) supported their recommendation and preferred elective treatment for N0 neck,(Andersen *et al.* 1996; Bourgier *et al.* 2005; Brazilian 1998; Byers *et al.* 1998; Dias *et al.* 2001; Ferlito *et al.* 2006; Franceschi *et al.* 1993; Greenberg *et al.* 2003; Haddadin *et al.* 1999; Huang *et al.* 2008; Kaya *et al.* 2001; O'brien *et al.* 2000; Sano and Myers 2007; Wei *et al.* 2006; Yuen *et al.* 1997) because their occult metastatic rates were much higher than 20% (Table 2). Currently, the National Cancer Comprehensive Network's adopted practice guidelines have recommended elective neck dissection for clinical N0 cancer of the oral cavity, oropharynx, hypopharynx and supraglottic larynx. (NCCN, 2011) These guidelines apply to the performance of

elective neck dissections as part of treatment of the primary tumor.

Another reason of the preference for elective neck dissection is less morbidity of supraomoyhoid neck dissection (SOHND) compared to classical radical neck dissection.(Spiro *et al.* 1996) For primary tumors in the oral cavity the regional lymph nodes at highest risk for early dissemination by metastatic cancer are limited to Levels I, II, and III

Pathologically positive

CT MRI US

metastasis. The five-year relative survival rate of patients who present with tumors localized at the primary site without dissemination to regional lymph nodes is 82%.(SEER, Oral cancer statistics, http://seer.cancer.gov/statfacts/html/oralcav.html) On the other hand, once dissemination to regional lymph nodes takes place, the survival rate reduces to nearly 50%. Advancement of regional disease, such as extracapsular spread and multiple nodal metastases, has influenced survival.(Myers *et al.* 2001; Shaw *et al.* 2009) Clearly, the regional status is the most significant independent prognostic factor,(Okura 2002; Shah *et al.* 1993; Taniguchi and Okura 2003) and appropriate management of the cervical lymph nodes is essential for control of disease.(Ferlito *et al.* 2006) In this chapter, we review the literature to ascertain whether elective neck dissection should be performed for cN0 neck or wait-and-see policy is safe and adequate.

#### **1.1 Treatment of clinical N0 (cN0) neck**

The management of neck disease in head and neck cancer, including oral cavity cancer, has been considered one of the most important aspects of treatment. When nodal metastases are present, nobody can deny the important effect of therapeutic neck dissection in the prognosis of head and neck cancer patients. However, the role of elective neck dissection has been a matter of discussion. Even the patients with clinically negative nodes (cN0) may still harbor occult metastasis, although advances in imaging techniques such as computed tomography, magnetic resonance, ultrasound sonography (US), and positron emission tomography have increased the accuracy of nodal metastases (Figure 2).

Fig. 2. Imagings of a patient with T2N0M0 SCC of the oral tongue.

A, preoperative positron emission tomography; B, preoperative enhanced computer tomography; C, enhanced computer tomography two months after transoral excision of the tumor. Preoperative assessment shows no involved node in the neck (A, B). Note late cervical nodal metastasis in the right side of the neck (Level III, C).

Table 1 shows the results of neck metastases and of each imaging study per patient with SCC of the oral tongue. CT had 70% accuracy, MRI had 74%, and US had 83% accuracy. Among the three image techniques US had the highest accuracy, although the accuracy was dependent on the observer. Our policy is essentially wait-and-see, and most of falsenegative nodes were detected within 12 months after the initial transoral excision. In this study of oral tongue 66 (69%) patients with cN0 neck received intraoral excison alone, and 16 (17%) developed late lymph node metastases. The rate of occult metastases was 17% for SCC of the oral tongue and 21% for SCC of the oral cavity. Since the occult metastatic rate of head and neck cancer ranges from 17—50% (average, 28%) in the literature (Table 2), the optimal method of management of clinical N0 neck remains controversial.


metastasis. The five-year relative survival rate of patients who present with tumors localized at the primary site without dissemination to regional lymph nodes is 82%.(SEER, Oral cancer statistics, http://seer.cancer.gov/statfacts/html/oralcav.html) On the other hand, once dissemination to regional lymph nodes takes place, the survival rate reduces to nearly 50%. Advancement of regional disease, such as extracapsular spread and multiple nodal metastases, has influenced survival.(Myers *et al.* 2001; Shaw *et al.* 2009) Clearly, the regional status is the most significant independent prognostic factor,(Okura 2002; Shah *et al.* 1993; Taniguchi and Okura 2003) and appropriate management of the cervical lymph nodes is essential for control of disease.(Ferlito *et al.* 2006) In this chapter, we review the literature to ascertain whether elective neck dissection should be performed for cN0 neck or wait-and-see policy is safe and adequate.

The management of neck disease in head and neck cancer, including oral cavity cancer, has been considered one of the most important aspects of treatment. When nodal metastases are present, nobody can deny the important effect of therapeutic neck dissection in the prognosis of head and neck cancer patients. However, the role of elective neck dissection has been a matter of discussion. Even the patients with clinically negative nodes (cN0) may still harbor occult metastasis, although advances in imaging techniques such as computed tomography, magnetic resonance, ultrasound sonography (US), and positron emission

tomography have increased the accuracy of nodal metastases (Figure 2).

Fig. 2. Imagings of a patient with T2N0M0 SCC of the oral tongue.

cervical nodal metastasis in the right side of the neck (Level III, C).

optimal method of management of clinical N0 neck remains controversial.

A, preoperative positron emission tomography; B, preoperative enhanced computer tomography; C, enhanced computer tomography two months after transoral excision of the tumor. Preoperative assessment shows no involved node in the neck (A, B). Note late

Table 1 shows the results of neck metastases and of each imaging study per patient with SCC of the oral tongue. CT had 70% accuracy, MRI had 74%, and US had 83% accuracy. Among the three image techniques US had the highest accuracy, although the accuracy was dependent on the observer. Our policy is essentially wait-and-see, and most of falsenegative nodes were detected within 12 months after the initial transoral excision. In this study of oral tongue 66 (69%) patients with cN0 neck received intraoral excison alone, and 16 (17%) developed late lymph node metastases. The rate of occult metastases was 17% for SCC of the oral tongue and 21% for SCC of the oral cavity. Since the occult metastatic rate of head and neck cancer ranges from 17—50% (average, 28%) in the literature (Table 2), the

**1.1 Treatment of clinical N0 (cN0) neck** 


Table 1. Image accuracy of nodal positivity in patients with SCC of the oral tongue. 1Pathologically negative includes patients who performed intraoral excision alone with no regional recurrence; CT, computed tomography; MRI, magnetic resonance imaging; US, ultrasound sonography; -, negative; +, positive. Number indicates patient number.

The N0 neck can be treated electively or can be carefully observed (wait-and-see), and the decision can be made from each own clinical experience (Table 1). Randomized controlled trial (RCT) is desired to determine which is preferred, however RCT is not easy task. So far four RCTs of the small sample size had been performed. Vandenbrouck et al.(Vandenbrouck *et al.* 1980) demonstrated that the survival rates were similar between two treatment arms in 75 patients with oral cavity cancer, whereas Fakih et al.(Fakih *et al.* 1989) (n = 70) and Klingerman et al.(Kligerman *et al.* 1994) (n = 67) found that elective neck dissection had significant benefit for patients with tumor thickness of more than 4 mm. In 2009, Yuen et al.(Yuen *et al.* 2009) demonstrated that disease-free survival was quite similar between two arms in 71 patients with SCC of the oral tongue. Thus, these four RCTs failed to impact on clinicians due to the inconsistency and small number of cases studied. In 1994 Weiss et al.(Weiss *et al.* 1994) created a decision tree analysis and demonstrated that when the probability of occult cervical metastasis is more than 20%, the neck should be electively treated. Since then a large number of studies(Dias *et al.* 2001; Ferlito *et al.* 2006; Greenberg *et al.* 2003; Haddadin *et al.* 1999; O'brien *et al.* 2000; Sano and Myers 2007; Wei *et al.* 2006) supported their recommendation and preferred elective treatment for N0 neck,(Andersen *et al.* 1996; Bourgier *et al.* 2005; Brazilian 1998; Byers *et al.* 1998; Dias *et al.* 2001; Ferlito *et al.* 2006; Franceschi *et al.* 1993; Greenberg *et al.* 2003; Haddadin *et al.* 1999; Huang *et al.* 2008; Kaya *et al.* 2001; O'brien *et al.* 2000; Sano and Myers 2007; Wei *et al.* 2006; Yuen *et al.* 1997) because their occult metastatic rates were much higher than 20% (Table 2). Currently, the National Cancer Comprehensive Network's adopted practice guidelines have recommended elective neck dissection for clinical N0 cancer of the oral cavity, oropharynx, hypopharynx and supraglottic larynx. (NCCN, 2011) These guidelines apply to the performance of elective neck dissections as part of treatment of the primary tumor.

Another reason of the preference for elective neck dissection is less morbidity of supraomoyhoid neck dissection (SOHND) compared to classical radical neck dissection.(Spiro *et al.* 1996) For primary tumors in the oral cavity the regional lymph nodes at highest risk for early dissemination by metastatic cancer are limited to Levels I, II, and III

Management for the N0 Neck of SCC in the Oral Cavity 117

involvement, complications of treatment, and disease control rates.(Weiss *et al.* 1994) In sensitivity analysis they defined the expected utility to a function of the occult metastatic rate. They concluded that cN0 necks should be treated electively when the occult metastatic rate is more than 20%. The 20% of threshold has likely exerted a great influence on the management of cN0 necks, because they estimated curable probabilities using data of reviews published in the 1980s. Weiss et al. have however alluded that the values will change and the threshold will be altered with the times. The recommendation for elective neck dissection in more than two decades has to be reconsidered with the current data. Accordingly we have reconfigured the decision tree sensitivity analysis with our current disease control rates to determine optimal therapy based on a current set of underlying assumptions.(Okura *et al.* 2009) Two decision tree strategies for the management of cN0 neck was compared; elective neck dissection or wait-and-see. In sensitivity analysis the expected utility for each strategy is a function of occult metastatic rate according to Weiss study. The higher utility value is preferable to the lower

The treatment threshold between elective neck dissection and observation was estimated with three (*a–c*) probabilities of survival; *a* = the curable probability (5-year overall survival rate) of the patients received elective neck dissection with no neck recurrence, *b* = the curable probability of the observed patients with late neck metastasis, *c* = the curable probability of the observed patients with no neck recurrence. These three probabilities are different in each institution. With the sensitivity analysis, the treatment threshold (Rx) can

Rx = (*c* − 0.97*a*) (0.00376 − 0.0776*a* − 0.94*b* + *c*). When clinicians calculate their own 3 probabilities (*a–c*) of being cured, they can estimate their own threshold for treatment of cN0 neck using this formula. The formula will be put to practical use and will estimate the current threshold. Our calculated threshold of the occult rate between the two strategies was 44.4% (Table 3). In our practice a patient with SCC of the oral cavity and N0 neck should be carefully observed if the probability of occult cervical metastasis is less than 44.4%. Only if the probability is greater than 44.4%, elective neck dissection might be warranted. Since the probability *c* is the survival rate for patients with no involved nodes who do not have occult metastases, c is expected to be a high rate. The probability *a* is the survival rate for patients who received elective neck dissection and should be lower than *c,* because some have occult metastases. If the occult metastatic rate is 0%, then the probability *a* would be quite same to *c*. A high occult metastatic rate and poor survival for patients with occult metastases contribute to a difference in probability between *a* and *c*. Table 3 shows the treatment threshold in various three probabilities according to the formula. For instance, assuming that *c* is fixed to 80% and *a* is 65% gives Rx more than 30% when *b* is not less than 20%. Rx becomes greater in proportion to the increase of *b*, because the denominator in the formula is decreased. Assuming that *a* is 70%, Rx is more than 22%. Assuming that the difference between *a* and *c* is 5% (*a* = 75%), Rx is 13% when *b* = 20% and 16% when *b* = 30%, respectively. These Rx rates are too lower, however Rx goes up to more than 30% when *b* is more than 54%. Furthermore, providing that the difference between *a* and *c* is decreased to 2%, Rx is below 20% when *b* is less than 50%. For giving Rx > 30%, *b* needs more than 64%. It is therefore necessary for giving Rx high percentage to build up

one, and the intersection indicates the treatment threshold.

be calculated through the following:

**1.3 Formula of the threshold for the treatment of cN0 neck** 


Table 2. Analysis of occult metastasis.

OT, oral tongue; OC, oral cavity; OP, oropharynx; H&N, head and neck; obs, observation; END, elective neck dissection; SOHND, supraomohyoid neck dissection; mRND, modified radical neck dissection.

in the supraomohyoid triangle. Skip metastasis to Levels IV and V in the absence of metastatic disease at Levels I, II, or III is exceedingly rare.(Shah *et al.* 1993) Compared to radical neck dissection SOHND reduces morbidity, including spinal accessory nerve disorder which results in diminished or absent function of the sternocleidomastoid muscle and upper portion of the trapezius muscle, and reduces cosmetic deformity. In addition, SOHND is considered as effective as comprehensive procedures for staging the clinically negative neck, when the neck is treated electively. It is intrinsic in the philosophy of a preventive treatment, to make it the less morbidity possible without losing oncologic results. However, this elective policy results in overtreatment of the neck, when the neck actually has no involved nodes. The less shoulder morbidity accompanied with SOHND is nonzero. Approximately 20% of patients who received SOHND had a shoulder pain even with conserving the accessary nerve.(Van Wilgen *et al.* 2004) Such overtreatment should be avoided when patients have no involved nodes in the neck.

#### **1.2 Decision tree analysis**

Upon returning decision tree analysis of Weiss et al., the decision tree is based on an analysis of the utility of the management options taking into account the incidence of node

Patient number % of occult

metastasis Neck treatment

T stage

Ho, 1992 OT T1-2 28 42 obs Lim, 2004 OT T1-2 56 32 obs Goto, 2005 OT T1-2 88 26 obs, END Lim, 2006 OT T1-2 54 28 obs, SOHND Keski-Sӓntti, 2006 OT T1-2 80 30 obs, END Kligerman, 1994 OC T1-2 67 43 obs, SOHND Brazilian H&N, 1998 OC T2-4 148 28 mRND, SOHND Kaneko, 2002 OC T1-4 868 17 obs, END Amaral, 2004 OC T1-2 117 23 END Smith, 2004 OC T1-2 150 28 obs, END Zbӓren, 2006 OC T1-3 100 20 SOHND Clark, 2006 OC T1-4 105 34 obs, END Mathew lype, 2008 OC T1-4 219 27 SOHND Okura, 2009 OC T1-4 165 21 obs, END Kraus, 1996 OC, OP T1-4 44 32 SOHND Nieuwenhuis, 2001 OC, OP T1-2 161 21 obs Duvvuri, 2004 OC, OP T1-2 359 25 obs, END O'Brien, 2008 OC, OP T1-4 108 30 END Spiro, 1996 H&N - 268 25 SOHND van den Brekel, 1999 H&N T1-4 77 18 obs Coatesworth, 2002 H&N T1-4 63 30 END Gourin, 2008 H&N T1-4 337 50 END

Author, year Primary

site

Total 3662 28

avoided when patients have no involved nodes in the neck.

OT, oral tongue; OC, oral cavity; OP, oropharynx; H&N, head and neck; obs, observation; END, elective neck dissection; SOHND, supraomohyoid neck dissection; mRND, modified

in the supraomohyoid triangle. Skip metastasis to Levels IV and V in the absence of metastatic disease at Levels I, II, or III is exceedingly rare.(Shah *et al.* 1993) Compared to radical neck dissection SOHND reduces morbidity, including spinal accessory nerve disorder which results in diminished or absent function of the sternocleidomastoid muscle and upper portion of the trapezius muscle, and reduces cosmetic deformity. In addition, SOHND is considered as effective as comprehensive procedures for staging the clinically negative neck, when the neck is treated electively. It is intrinsic in the philosophy of a preventive treatment, to make it the less morbidity possible without losing oncologic results. However, this elective policy results in overtreatment of the neck, when the neck actually has no involved nodes. The less shoulder morbidity accompanied with SOHND is nonzero. Approximately 20% of patients who received SOHND had a shoulder pain even with conserving the accessary nerve.(Van Wilgen *et al.* 2004) Such overtreatment should be

Upon returning decision tree analysis of Weiss et al., the decision tree is based on an analysis of the utility of the management options taking into account the incidence of node

Table 2. Analysis of occult metastasis.

radical neck dissection.

**1.2 Decision tree analysis** 

involvement, complications of treatment, and disease control rates.(Weiss *et al.* 1994) In sensitivity analysis they defined the expected utility to a function of the occult metastatic rate. They concluded that cN0 necks should be treated electively when the occult metastatic rate is more than 20%. The 20% of threshold has likely exerted a great influence on the management of cN0 necks, because they estimated curable probabilities using data of reviews published in the 1980s. Weiss et al. have however alluded that the values will change and the threshold will be altered with the times. The recommendation for elective neck dissection in more than two decades has to be reconsidered with the current data. Accordingly we have reconfigured the decision tree sensitivity analysis with our current disease control rates to determine optimal therapy based on a current set of underlying assumptions.(Okura *et al.* 2009) Two decision tree strategies for the management of cN0 neck was compared; elective neck dissection or wait-and-see. In sensitivity analysis the expected utility for each strategy is a function of occult metastatic rate according to Weiss study. The higher utility value is preferable to the lower one, and the intersection indicates the treatment threshold.

#### **1.3 Formula of the threshold for the treatment of cN0 neck**

The treatment threshold between elective neck dissection and observation was estimated with three (*a–c*) probabilities of survival; *a* = the curable probability (5-year overall survival rate) of the patients received elective neck dissection with no neck recurrence, *b* = the curable probability of the observed patients with late neck metastasis, *c* = the curable probability of the observed patients with no neck recurrence. These three probabilities are different in each institution. With the sensitivity analysis, the treatment threshold (Rx) can be calculated through the following:

$$\mathbf{R}\mathbf{x} = (\mathbf{c} - 0.97a) / (0.00376 - 0.0776a - 0.94b + c).$$

When clinicians calculate their own 3 probabilities (*a–c*) of being cured, they can estimate their own threshold for treatment of cN0 neck using this formula. The formula will be put to practical use and will estimate the current threshold. Our calculated threshold of the occult rate between the two strategies was 44.4% (Table 3). In our practice a patient with SCC of the oral cavity and N0 neck should be carefully observed if the probability of occult cervical metastasis is less than 44.4%. Only if the probability is greater than 44.4%, elective neck dissection might be warranted. Since the probability *c* is the survival rate for patients with no involved nodes who do not have occult metastases, c is expected to be a high rate. The probability *a* is the survival rate for patients who received elective neck dissection and should be lower than *c,* because some have occult metastases. If the occult metastatic rate is 0%, then the probability *a* would be quite same to *c*. A high occult metastatic rate and poor survival for patients with occult metastases contribute to a difference in probability between *a* and *c*.

Table 3 shows the treatment threshold in various three probabilities according to the formula. For instance, assuming that *c* is fixed to 80% and *a* is 65% gives Rx more than 30% when *b* is not less than 20%. Rx becomes greater in proportion to the increase of *b*, because the denominator in the formula is decreased. Assuming that *a* is 70%, Rx is more than 22%. Assuming that the difference between *a* and *c* is 5% (*a* = 75%), Rx is 13% when *b* = 20% and 16% when *b* = 30%, respectively. These Rx rates are too lower, however Rx goes up to more than 30% when *b* is more than 54%. Furthermore, providing that the difference between *a* and *c* is decreased to 2%, Rx is below 20% when *b* is less than 50%. For giving Rx > 30%, *b* needs more than 64%. It is therefore necessary for giving Rx high percentage to build up

Management for the N0 Neck of SCC in the Oral Cavity 119

The rate is increased in proportion of the increase of T stage, except for T4. The increase of primary lesions compels us to consider elective treatment, although the highest rate for T3 is still lower than our treatment threshold (44.4%). Other predictors of occult metastases are

Numerous studies have reported that histologic tumor thickness correlates closely with lymph node metastases in SCC of the oral cavity.(Asakage *et al.* 1998; Byers *et al.* 1998; Fukano *et al.* 1997; Lim *et al.* 2004; O-Charoenrat *et al.* 2003; Spiro *et al.* 1986; Yamazaki *et al.* 2004; Yuen *et al.* 2000) Patients with more than 3 – 6 mm of histologic tumor thickness recommends to treated electively because of high risk of metastases. However accurate preoperative assessment of the thickness in biopsy section is no easy task. It is occasionally difficult to reach an invasive front on biopsy, and the tumor thickness on biopsy is not necessarily the greatest. In order to detect tumor thickness more accurately, sequential sections are desirable but not pragmatic. Accordingly, multi-sliced imaging techniques

Recently, the correlation between histologic tumor thickness and magnetic resonance imaging (MRI) tumor thickness was demonstrated (Iwai *et al.* 2002; Lam *et al.* 2004; Preda *et al.* 2006). Then MRI tumor thickness seems to become a candidate of occult metastatic predictor, although these studies did not reach to demonstrate the relation with MRI tumor thickness and regional metastases. We have verified MRI tumor thickness in patients with oral tongue SCC.(Okura *et al.* 2008) Coronal MRI was preferred to measure tumor thickness

Fig. 3. Coronal contrasted-enhanced T1-weighted MRI shows tumor thickness (T) and paralingual distance (P). A vertical white line is a reference line connecting two tumormucosa junctions. A horizontal white line drawn perpendicular to the reference line

represents radiologically is tumor thickness (T). The image shows that a high-intensity area, paralingual spatium, extends from the medial border of the sublingual space to the deep lingual artery along the genioglossus. The white line (P) is the paralingual distance between

essential to management of cN0 necks.

should be useful and convenient.

than axial image (Figure 3).

the tumor and the paralingual spatium.


high *b* probability as well as the positive difference between *a* and *c*. First, we have to raise the successful salvage rate for patients with late neck metastases. In Table 3 when b is more than 60%, Rx is invariably over 24%.

Table 3. Three probabilities (*a*–*c*) and treatment threshold (Rx).

Rx of our study (2009) was two times or more as high as that of Weiss study (1994). Rx is calculated with each *a, b* and *c* using the formula.

#### **1.4 Predictors of occult metastases**

The management of cN0 neck of SCC of the oral cavity is not necessarily wait-and-see (observation). In our study the overall occult rate was 21%, relatively low compared to other studies (Table 2) and our policy is wait-and-see. Notwithstanding, one-fifth of patients with cN0 neck need late neck dissection when all necks of those patients were observed. Patients with higher probability of occult metastases are encouraged to be selected with other predictors. For instance, our occult metastatic rate was 14% for T1 lesions, 23% for T2 lesions, and 30% for T3 lesions, respectively (Table 4).


Table 4. Incidence and rate of occult metastases according to T stage.

high *b* probability as well as the positive difference between *a* and *c*. First, we have to raise the successful salvage rate for patients with late neck metastases. In Table 3 when b is more

> 60% 50% 60% 21% Weiss et al. 87.8% 71.3% 94.5% 44.4% Okura et al.

*a b c* Rx

65% 20% 80% 30% 65% 30% 80% 36% 70% 20% 80% 22% 70% 30% 80% 26% 70% 40% 80% 32% 70% 50% 80% 43% 75% 20% 80% 13% 75% 30% 80% 16% 75% 40% 80% 20% 75% 54% 80% 30% 75% 60% 80% 40% 78% 30% 80% 9% 78% 40% 80% 12% 78% 50% 80% 16% 78% 60% 80% 24% 78% 64% 80% 31% 78% 70% 80% 51%

Table 3. Three probabilities (*a*–*c*) and treatment threshold (Rx).

calculated with each *a, b* and *c* using the formula.

lesions, and 30% for T3 lesions, respectively (Table 4).

Table 4. Incidence and rate of occult metastases according to T stage.

**1.4 Predictors of occult metastases** 

Rx of our study (2009) was two times or more as high as that of Weiss study (1994). Rx is

The management of cN0 neck of SCC of the oral cavity is not necessarily wait-and-see (observation). In our study the overall occult rate was 21%, relatively low compared to other studies (Table 2) and our policy is wait-and-see. Notwithstanding, one-fifth of patients with cN0 neck need late neck dissection when all necks of those patients were observed. Patients with higher probability of occult metastases are encouraged to be selected with other predictors. For instance, our occult metastatic rate was 14% for T1 lesions, 23% for T2

> T stage Incidence Rate T1 21/152 14% T2 54/232 23% T3 16/53 30% T4 7/38 18% Total 98/475 21%

than 60%, Rx is invariably over 24%.

The rate is increased in proportion of the increase of T stage, except for T4. The increase of primary lesions compels us to consider elective treatment, although the highest rate for T3 is still lower than our treatment threshold (44.4%). Other predictors of occult metastases are essential to management of cN0 necks.

Numerous studies have reported that histologic tumor thickness correlates closely with lymph node metastases in SCC of the oral cavity.(Asakage *et al.* 1998; Byers *et al.* 1998; Fukano *et al.* 1997; Lim *et al.* 2004; O-Charoenrat *et al.* 2003; Spiro *et al.* 1986; Yamazaki *et al.* 2004; Yuen *et al.* 2000) Patients with more than 3 – 6 mm of histologic tumor thickness recommends to treated electively because of high risk of metastases. However accurate preoperative assessment of the thickness in biopsy section is no easy task. It is occasionally difficult to reach an invasive front on biopsy, and the tumor thickness on biopsy is not necessarily the greatest. In order to detect tumor thickness more accurately, sequential sections are desirable but not pragmatic. Accordingly, multi-sliced imaging techniques should be useful and convenient.

Recently, the correlation between histologic tumor thickness and magnetic resonance imaging (MRI) tumor thickness was demonstrated (Iwai *et al.* 2002; Lam *et al.* 2004; Preda *et al.* 2006). Then MRI tumor thickness seems to become a candidate of occult metastatic predictor, although these studies did not reach to demonstrate the relation with MRI tumor thickness and regional metastases. We have verified MRI tumor thickness in patients with oral tongue SCC.(Okura *et al.* 2008) Coronal MRI was preferred to measure tumor thickness than axial image (Figure 3).

Fig. 3. Coronal contrasted-enhanced T1-weighted MRI shows tumor thickness (T) and paralingual distance (P). A vertical white line is a reference line connecting two tumormucosa junctions. A horizontal white line drawn perpendicular to the reference line represents radiologically is tumor thickness (T). The image shows that a high-intensity area, paralingual spatium, extends from the medial border of the sublingual space to the deep lingual artery along the genioglossus. The white line (P) is the paralingual distance between the tumor and the paralingual spatium.

Management for the N0 Neck of SCC in the Oral Cavity 121

Clinical N0 necks were treated with two treatment arms: observation and elective neck

The outcome depends on the extent of the occult metastases at the time they are detected, which correlates with the intensity of follow-up.(Cheng and Schmidt 2008) In our practice, follow-up took place at every month in the first year, at two months in the second year, at three months in the third year, at four months in the fourth year, and 6 months in the fifth year. CT scan and ultrasound sonography were performed every half year. The follow-up is basic management, and the optional examinations are appended individually. For instance, patients with higher risk of lymph node metastases have ultrasound examination more times, and patients with higher risk of distant metastases have positron emission tomography test or pulmonary CT scan. It is important to understand which risks are high.

At present, it is impossible to set the incidence of occult metastases to zero. Additionally, more sensitive parameters or markers associated with the presence of nodal metastasis are encouraged to be developed. The continued advancement will have a significant impact on the evaluation, management and outcome of patients with the oral cavity. For the current management for the cN0 neck, the important points are: Clinicians have to comprehend their own threshold between observation and elective neck dissection. For that purpose, it is necessary to estimate the occult metastatic rate and three probabilities of survival (*a, b, c*). Then, the best policy of the management of cN0 necks is able to be controlled and determined. Extended operations with less morbidity in surgical oncology have been pursued to improve the outcomes. However, these extended operations are not necessarily wise. Recently, extended para-aortic nodal dissection did not improve the survival of patients with gastric cancer,(Sasako *et al.* 2008) and axillary lymph node dissection should be considered unnecessary for woman with T1-2 invasive breast cancer.(Giuliano *et al.* 2011)

In SCC of the oral cavity, elective neck dissection does not also seem to be superior to a wait-and-see policy, and vice versa. Current studies, retrospective and prospective, have been unable to give us definitive recommendations regarding the management of the cN0

Thus, some extended operations do not seem to be the standard treatment.

Fig. 4. Overall survival according to clinical N stage.

dissection.

**3. Conclusion** 

Eighty-one % MRI permitted us the measurement of tumor thickness, however the remaining 19% could not be interpreted because of the interference of artifacts. There are some patients unsuitable for MR scan. Notwithstanding, MRI tumor thickness was related to lymph node metastases, and the mean of tumor thickness in patients with nodal metastases was twice length of that without nodal metastases. The thicker tumor thickness is, the higher the probability of lymph node metastases is (Table 5). Using logistic regression model, MRI tumor thickness was able to predict nodal metastasis in SCC of the oral tongue. Multivariate logistic regression function showed that if tumor thickness was 9.7mm, then the probalility of metastases was 20%. Tongue cancer varies in the growth pattern, endophytic or exophytic. Even if tumor thickness is similar in size, the position of the invasive front is different between endophytic and exophytic tumors. In order to observe where tumor cells invade, the paralingual distance between the invasive front and the paralingual spatium of tongue was measured (Figure 3). The paralingual spatium is loose connective tissue, which locates between the genioglossus muscle and the intrinsic tongue muscles to sublingual space. Lingual nerve and lingual artery run through this spatium, and the lingual artery is the landmark of this spatium. The paraligual distance was significantly related to lymph node metastases. The probability of nodal metastasis was in inverse proportion to the paralingual distance, and the probability was 20% at 5.2 mm of paralingual distance (Table 5). The two MRI parameters were more reliable than preoperative assessment of clinical N staging because of the log likelihood ratio. In our practice, when MR tumor thickness is more than 9.7 mm or paralingual distance is less than 5.2mm, we take elective neck dissection into consideration.


Table 5. Lymph node metastasis and measured MRI distances in SCC of the oral tongue.

Entering 5.2 mm of paralingual distance into the cut-off point resulted in 82% of specificity, 70% of sensitivity, and 14% of occult metastatic rate. The specificity of paralingual distance is higher than that using other images and the occult metastatic rate is the lowest (Table 1). Thus, MRI distances are useful to detect occult metastases of the oral tongue. Other endeavor to improve the accuracy of detecting occult metastases will be required.

#### **2. Survival**

In our study overall survival was similar, whether patients with cN0 neck are observed or electively treated. The 5-year overall survival rate for observed patients was 89%, and the rate for patients received elective neck dissection was 86% (Figure 4). On the other hand, patients with cN 1-3 neck had significantly lower overall survival (54% at 5-year) than those with cN0 neck.

Eighty-one % MRI permitted us the measurement of tumor thickness, however the remaining 19% could not be interpreted because of the interference of artifacts. There are some patients unsuitable for MR scan. Notwithstanding, MRI tumor thickness was related to lymph node metastases, and the mean of tumor thickness in patients with nodal metastases was twice length of that without nodal metastases. The thicker tumor thickness is, the higher the probability of lymph node metastases is (Table 5). Using logistic regression model, MRI tumor thickness was able to predict nodal metastasis in SCC of the oral tongue. Multivariate logistic regression function showed that if tumor thickness was 9.7mm, then the probalility of metastases was 20%. Tongue cancer varies in the growth pattern, endophytic or exophytic. Even if tumor thickness is similar in size, the position of the invasive front is different between endophytic and exophytic tumors. In order to observe where tumor cells invade, the paralingual distance between the invasive front and the paralingual spatium of tongue was measured (Figure 3). The paralingual spatium is loose connective tissue, which locates between the genioglossus muscle and the intrinsic tongue muscles to sublingual space. Lingual nerve and lingual artery run through this spatium, and the lingual artery is the landmark of this spatium. The paraligual distance was significantly related to lymph node metastases. The probability of nodal metastasis was in inverse proportion to the paralingual distance, and the probability was 20% at 5.2 mm of paralingual distance (Table 5). The two MRI parameters were more reliable than preoperative assessment of clinical N staging because of the log likelihood ratio. In our practice, when MR tumor thickness is more than 9.7 mm or paralingual distance is less than

> Probability of lymph Tumor Paralingual node metastasis thickness distance (%) (mm) (mm) 10% 7.1 6.5 20% 9.7 5.2 25% 10.6 4.7 30% 11.5 4.3 40% 12.9 3.6 50% 14.2 3.0

Table 5. Lymph node metastasis and measured MRI distances in SCC of the oral tongue.

endeavor to improve the accuracy of detecting occult metastases will be required.

Entering 5.2 mm of paralingual distance into the cut-off point resulted in 82% of specificity, 70% of sensitivity, and 14% of occult metastatic rate. The specificity of paralingual distance is higher than that using other images and the occult metastatic rate is the lowest (Table 1). Thus, MRI distances are useful to detect occult metastases of the oral tongue. Other

In our study overall survival was similar, whether patients with cN0 neck are observed or electively treated. The 5-year overall survival rate for observed patients was 89%, and the rate for patients received elective neck dissection was 86% (Figure 4). On the other hand, patients with cN 1-3 neck had significantly lower overall survival (54% at 5-year) than those

5.2mm, we take elective neck dissection into consideration.

**2. Survival** 

with cN0 neck.

Fig. 4. Overall survival according to clinical N stage. Clinical N0 necks were treated with two treatment arms: observation and elective neck dissection.

The outcome depends on the extent of the occult metastases at the time they are detected, which correlates with the intensity of follow-up.(Cheng and Schmidt 2008) In our practice, follow-up took place at every month in the first year, at two months in the second year, at three months in the third year, at four months in the fourth year, and 6 months in the fifth year. CT scan and ultrasound sonography were performed every half year. The follow-up is basic management, and the optional examinations are appended individually. For instance, patients with higher risk of lymph node metastases have ultrasound examination more times, and patients with higher risk of distant metastases have positron emission tomography test or pulmonary CT scan. It is important to understand which risks are high.

#### **3. Conclusion**

At present, it is impossible to set the incidence of occult metastases to zero. Additionally, more sensitive parameters or markers associated with the presence of nodal metastasis are encouraged to be developed. The continued advancement will have a significant impact on the evaluation, management and outcome of patients with the oral cavity. For the current management for the cN0 neck, the important points are: Clinicians have to comprehend their own threshold between observation and elective neck dissection. For that purpose, it is necessary to estimate the occult metastatic rate and three probabilities of survival (*a, b, c*). Then, the best policy of the management of cN0 necks is able to be controlled and determined.

Extended operations with less morbidity in surgical oncology have been pursued to improve the outcomes. However, these extended operations are not necessarily wise. Recently, extended para-aortic nodal dissection did not improve the survival of patients with gastric cancer,(Sasako *et al.* 2008) and axillary lymph node dissection should be considered unnecessary for woman with T1-2 invasive breast cancer.(Giuliano *et al.* 2011) Thus, some extended operations do not seem to be the standard treatment.

In SCC of the oral cavity, elective neck dissection does not also seem to be superior to a wait-and-see policy, and vice versa. Current studies, retrospective and prospective, have been unable to give us definitive recommendations regarding the management of the cN0

Management for the N0 Neck of SCC in the Oral Cavity 123

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#### **4. Acknowledgment**

This work was conducted at the first department of Oral & Maxillofacial Surgery, Osaka University Dental Hospital in Japan. This work is supported in part by Grants from Ministry of Education, Science and Culture, Japan. We would like to express great gratitude to Chief professor Mikihiko Kogo and all clinical members in Osaka University Dental Hospital. We have furthermore to thank our secretary, Ms. Aya Okano to support this work.

#### **5. References**


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http://www.nccn.org/professionals/physician\_gls/f\_guidelines.asp


**9** 

*Japan* 

Yuki Saito and Takahiro Asakage

*The University of Tokyo,* 

 *Department of Otolaryngology, Head and Neck Surgery,* 

**Neck Dissection of the Head and Neck Sarcoma** 

Sarcomas of the Head and Neck (SHN) are rare tumors of connective tissue origin that comprise less than 1% of head and neck malignancies. SHN account for approximately 5% to 10% of all sarcomas (Bruce, 2004). They arise in any soft tissue or osseous tissues of the region and may be found in patients of any age or gender. This chapter we will present the essential

Diagnosis of SHN is a diagnostic challenge. Although biopsy by FNA identified the correct specific sarcoma type in only 21% of cases, FNA led to an appropriate diagnostic biopsy in 88% (Costa et al., 1996). In only 7% of cases were the FNA findings misleading (benign or inflammatory) and in 5% of cases the reading was interpreted as inadequate. More recently, FNA biopsies of sarcoma were more often interpreted as showing a lower grade than that determined by final analysis of the fully resected specimen (Jones et al. 2002). Superficial and accessible lesions or lesions with inconclusive results from prior closed biopsy should be biopsied by incisional or excisional technique; fine needle aspiration or core needle biopsy should be reserved for deeper and less accessible lesions (Schalow & Broecker, 2003). Frequently the initial procedure for patients with a mass suspected to be Rabdomyosarcoma is a biopsy, usually open, which obtains an adequate specimen for pathological, biological and treatment protocol studies. There may be instances when core needle biopsy is appropriate, such as metastatic disease or small lesions in areas that will be treated primarily by chemotherapy and radiotherapy (Rodeberg et al, 2002). Since lymphoma and nasopharyngeal carcinoma would show the same signal intensity characteristics as sarcomas on magnetic

elements of diagnosis and resection or neck dissection of head and neck sarcoma.

resonance, tissue biopsy is imperative for correct diagnosis (Flympas et al. 2009).

The main therapy for soft tissue sarcomas of the head and neck is en-bloc resection with a margin of normal tissue. The adequacy of surgical resection seems to be a major determinant of outcome for sarcomas. The difficulty with en-bloc resection in these tumors relates to the proximity of important neurovascular structures and vital organs, as well as the cosmetic and functional morbidity resulting from such radical extirpative procedures. It is known that some soft-tissue sarcomas develop a pseudocapsule as a result of compression of adjacent tissues. This may obscure the true invasive nature of these neoplasms. Sarcomas tend to extend outside this pseudocapsule and invade tissues that may seem uninvolved during surgery. Because of the anatomical challenges of en-bloc resection for soft tissue sarcomas of the head and neck, positive margins should prompt the addition of adjuvant radiotherapy. Death from SHN is often a consequence of uncontrolled local disease as it is from metastases, unlike other sarcomas where local salvage options are often available.

**1. Introduction** 


### **Neck Dissection of the Head and Neck Sarcoma**

Yuki Saito and Takahiro Asakage

 *Department of Otolaryngology, Head and Neck Surgery, The University of Tokyo, Japan* 

#### **1. Introduction**

124 Neck Dissection – Clinical Application and Recent Advances

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0-7234-3223-6

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ISSN 1043-3074

paralingual distance of coronal MR imaging predicts cervical node metastases in

between histologic thickness of tongue carcinoma and thickness estimated from

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of tumor thickness in squamous carcinoma confined to the tongue and floor of the

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(2004) Shoulder and neck morbidity in quality of life after surgery for head and

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management strategy for the stage N0 neck. *Arch Otolaryngol Head Neck Surg* 120:

metastasis of early oral tongue cancer after interstitial radiotherapy. *Int J Radiat* 

randomized study of selective neck dissection versus observation for N0 neck of

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observation in the treatment of early oral tongue carcinoma. *Head Neck* 19: 583-588,

Sarcomas of the Head and Neck (SHN) are rare tumors of connective tissue origin that comprise less than 1% of head and neck malignancies. SHN account for approximately 5% to 10% of all sarcomas (Bruce, 2004). They arise in any soft tissue or osseous tissues of the region and may be found in patients of any age or gender. This chapter we will present the essential elements of diagnosis and resection or neck dissection of head and neck sarcoma.

Diagnosis of SHN is a diagnostic challenge. Although biopsy by FNA identified the correct specific sarcoma type in only 21% of cases, FNA led to an appropriate diagnostic biopsy in 88% (Costa et al., 1996). In only 7% of cases were the FNA findings misleading (benign or inflammatory) and in 5% of cases the reading was interpreted as inadequate. More recently, FNA biopsies of sarcoma were more often interpreted as showing a lower grade than that determined by final analysis of the fully resected specimen (Jones et al. 2002). Superficial and accessible lesions or lesions with inconclusive results from prior closed biopsy should be biopsied by incisional or excisional technique; fine needle aspiration or core needle biopsy should be reserved for deeper and less accessible lesions (Schalow & Broecker, 2003). Frequently the initial procedure for patients with a mass suspected to be Rabdomyosarcoma is a biopsy, usually open, which obtains an adequate specimen for pathological, biological and treatment protocol studies. There may be instances when core needle biopsy is appropriate, such as metastatic disease or small lesions in areas that will be treated primarily by chemotherapy and radiotherapy (Rodeberg et al, 2002). Since lymphoma and nasopharyngeal carcinoma would show the same signal intensity characteristics as sarcomas on magnetic resonance, tissue biopsy is imperative for correct diagnosis (Flympas et al. 2009).

The main therapy for soft tissue sarcomas of the head and neck is en-bloc resection with a margin of normal tissue. The adequacy of surgical resection seems to be a major determinant of outcome for sarcomas. The difficulty with en-bloc resection in these tumors relates to the proximity of important neurovascular structures and vital organs, as well as the cosmetic and functional morbidity resulting from such radical extirpative procedures. It is known that some soft-tissue sarcomas develop a pseudocapsule as a result of compression of adjacent tissues. This may obscure the true invasive nature of these neoplasms. Sarcomas tend to extend outside this pseudocapsule and invade tissues that may seem uninvolved during surgery. Because of the anatomical challenges of en-bloc resection for soft tissue sarcomas of the head and neck, positive margins should prompt the addition of adjuvant radiotherapy. Death from SHN is often a consequence of uncontrolled local disease as it is from metastases, unlike other sarcomas where local salvage options are often available.

Neck Dissection of the Head and Neck Sarcoma 127

pterygopalatine fossa. They represent a particular surgical challenge. Surgical excision may lead to unacceptable mutilations and incomplete resection in most cases. These tumours have a high likelihood of meningeal extension, which is considered a fatal pattern of progression. Most authors conclude that multi-agent chemotherapy combined with radiation is the primary treatment modality offered to children with PM-RMS. Surgery prior to chemotherapy is recommended if there is no intracranial extension, if complete resection seems feasible and if it

Head and neck RMS has proven able to spread to cervical lymph nodes. Comparing the two most frequent histologic subtypes i.e. embryonal and alveolar, the latter showed a higher tendency to metastasize through the lymphatic route. According to some authors, elective treatment of the neck is not justified. In view of the low incidence of cervical lymph node metastases of merely 3% according to the IRS (Intergroup Rhabdomyosarcoma Study), Rodeberg et al. saw no indication for conducting elective neck dissection (Rodeberg et al.2002). Wurm recommends elective neck dissection in patients with alveolar RMS when surgery on primary tumour is planned (Wurm, 2005). Callender et al. found regional metastasis in 15–38% of their patients respectively and inferred that elective neck dissection offers a benefit (Callender, 1995). Controversy exists also over treating the N+ neck with additional radiotherapy or neck dissection. If nodes are metastatic and complete primary tumour resection is feasible, neck dissection is recommended; while, if the lymph nodes are positive but the primary tumour is unresectable, they should be included in the radiotherapy field.

An 22 year-old female, who had been treated for retinoblastoma at six months of age, presented for evaluation of left temporal swelling. She had underwent enucleation of the bilateral eyeball and 40 Gy irradiation to the left orbit at 1 years of age. Examination revealed a rm mass overlying the left temporal muscle (Figure 1) and left cervical

Fig. 1. A 22-year-old female case of Rabdomyosarcoma, infantile type of the temporal muscle (arrow). She underwent the convination of chemotherapy and radiation 9 years ago,

now she was free of disease. (Our experience case)

won't lead to unacceptable morbidity. (Gradoni, et al. 2010)

**2.2 Lymphatic metastasis and neck dissection** 

**2.3 Case report** 

Most soft tissue sarcoma spread in a longitudinal direction within the muscle groups where they originate. They generally respect barriers to tumor spread, such as bones, interosseous membrane, major fascial planes, etc, and this feature should be exploited in planning tissue preserving approaches to management. As they extend, lesions invade muscle and contiguous structures or other vital anatomy (de Bree et al, 2010).

 Most SHN present with localized disease with metastasis being present in fewer than 10% of cases at the time of diagnosis and initial treatment. Predominant risk is to the lungs. Lymph node metastasis is considered an infrequent event in the natural history of soft tissue sarcomas. The rarity of such lymph node metastasis has made the study of their natural history difficult. Most published series are small, retrospective studies, and as individual studies are hard to interpret. The overall incidence of lymph node metastasis was estimated to be 10%. Lymphatic spread in sarcomas are rare, and that lymphatic spread is more frequently associated with certain histologic types. Those are, rhabdomyosarcomas, epithelioid sarcomas, angiosarcomas, and synovial sarcomas. Bone sarcomas may also present with regional lymph node disease, which carries a very adverse outcome. (Fong et al, 1993)

We discuss the previous reports of neck metastasis and neck dissection of main subtypes of Head and neck sarcoma.

1 Rabdomyosarcoma, 2 Fibrous tumor (desmoid tumor, fibrosarcoma, dermatofibrosarcoma protuberans), 3 Angiosarcoma 4 Synovial sarcoma, 5 Osteosarcoma, 6 Chondrosarcoma, 7 Ewing's sarcoma.

#### **2. Rabdomyosarcoma**

The rhabdomyosarcoma (RMS), a skeletal muscle subtype, is the most common soft tissue sarcoma in children, comprising about 50% of these tumors. In contrast, adult rhabdomyosarcomas comprise less than 10% of all soft tissue sarcomas. The head and neck are the most common primary sites for RMS in children and teenagers, followed by the genitourinary tract, limbs, thorax, and retroperitoneum. The tumor head and neck subsites include the orbit, parameningeal sites (nasopharynx, nasal cavity, paranasal sinuses, temporal bone, pterygopalatine fossa, and the infratemporal fossa), and non-parameningeal sites. Tumors that invade the orbit only have a better prognosis. (Moretti, 2010)

Subtypes may be histologically classified as: embryonic (which may be subdivided into embryonic, botryoid, and spindle cell tumors), alveolar, or pleomorphic. In children, about 60% are embryonic tumors, 20% are alveolar tumors, 15% are not classified, and 5% are pleomorphic tumors. The embryonic subtype has a better prognosis in children, but is more aggressive in adults. The alveolar subtype has a poor outcome because of its propensity to metastasize at a distance. The pleomorphic subtype occurs predominantly in adults. (World Health Organization Classification of Tumours [WHO] Tumors of Soft Tissue and Bone, 2010)

#### **2.1 Treatment**

Prior to the introduction of antineoplastic drugs, surgery played the central role in the cure of patients with RMS; in the last four decades, the development of multi-agent chemotherapy protocols resulted in a significant improvement in long-term survival: from 25% in 1970, to approximately 70% nowadays. In the head and neck area, a distinction is drawn between parameningeal rhabdomyosarcoma (PM-RMS) and RMS of other localizations. RM-RMS arises at sites with a particularly close anatomic relationship with the meninges such as nasopharynx, nasal cavity, paranasal sinuses, middle ear and mastoid, infratemporal and

Most soft tissue sarcoma spread in a longitudinal direction within the muscle groups where they originate. They generally respect barriers to tumor spread, such as bones, interosseous membrane, major fascial planes, etc, and this feature should be exploited in planning tissue preserving approaches to management. As they extend, lesions invade muscle and

 Most SHN present with localized disease with metastasis being present in fewer than 10% of cases at the time of diagnosis and initial treatment. Predominant risk is to the lungs. Lymph node metastasis is considered an infrequent event in the natural history of soft tissue sarcomas. The rarity of such lymph node metastasis has made the study of their natural history difficult. Most published series are small, retrospective studies, and as individual studies are hard to interpret. The overall incidence of lymph node metastasis was estimated to be 10%. Lymphatic spread in sarcomas are rare, and that lymphatic spread is more frequently associated with certain histologic types. Those are, rhabdomyosarcomas, epithelioid sarcomas, angiosarcomas, and synovial sarcomas. Bone sarcomas may also present with regional lymph node disease,

We discuss the previous reports of neck metastasis and neck dissection of main subtypes of

1 Rabdomyosarcoma, 2 Fibrous tumor (desmoid tumor, fibrosarcoma, dermatofibrosarcoma protuberans), 3 Angiosarcoma 4 Synovial sarcoma, 5 Osteosarcoma, 6 Chondrosarcoma, 7

The rhabdomyosarcoma (RMS), a skeletal muscle subtype, is the most common soft tissue sarcoma in children, comprising about 50% of these tumors. In contrast, adult rhabdomyosarcomas comprise less than 10% of all soft tissue sarcomas. The head and neck are the most common primary sites for RMS in children and teenagers, followed by the genitourinary tract, limbs, thorax, and retroperitoneum. The tumor head and neck subsites include the orbit, parameningeal sites (nasopharynx, nasal cavity, paranasal sinuses, temporal bone, pterygopalatine fossa, and the infratemporal fossa), and non-parameningeal

Subtypes may be histologically classified as: embryonic (which may be subdivided into embryonic, botryoid, and spindle cell tumors), alveolar, or pleomorphic. In children, about 60% are embryonic tumors, 20% are alveolar tumors, 15% are not classified, and 5% are pleomorphic tumors. The embryonic subtype has a better prognosis in children, but is more aggressive in adults. The alveolar subtype has a poor outcome because of its propensity to metastasize at a distance. The pleomorphic subtype occurs predominantly in adults. (World Health Organization Classification of Tumours [WHO] Tumors of Soft Tissue and Bone, 2010)

Prior to the introduction of antineoplastic drugs, surgery played the central role in the cure of patients with RMS; in the last four decades, the development of multi-agent chemotherapy protocols resulted in a significant improvement in long-term survival: from 25% in 1970, to approximately 70% nowadays. In the head and neck area, a distinction is drawn between parameningeal rhabdomyosarcoma (PM-RMS) and RMS of other localizations. RM-RMS arises at sites with a particularly close anatomic relationship with the meninges such as nasopharynx, nasal cavity, paranasal sinuses, middle ear and mastoid, infratemporal and

sites. Tumors that invade the orbit only have a better prognosis. (Moretti, 2010)

contiguous structures or other vital anatomy (de Bree et al, 2010).

which carries a very adverse outcome. (Fong et al, 1993)

Head and neck sarcoma.

**2. Rabdomyosarcoma** 

Ewing's sarcoma.

**2.1 Treatment** 

pterygopalatine fossa. They represent a particular surgical challenge. Surgical excision may lead to unacceptable mutilations and incomplete resection in most cases. These tumours have a high likelihood of meningeal extension, which is considered a fatal pattern of progression. Most authors conclude that multi-agent chemotherapy combined with radiation is the primary treatment modality offered to children with PM-RMS. Surgery prior to chemotherapy is recommended if there is no intracranial extension, if complete resection seems feasible and if it won't lead to unacceptable morbidity. (Gradoni, et al. 2010)

#### **2.2 Lymphatic metastasis and neck dissection**

Head and neck RMS has proven able to spread to cervical lymph nodes. Comparing the two most frequent histologic subtypes i.e. embryonal and alveolar, the latter showed a higher tendency to metastasize through the lymphatic route. According to some authors, elective treatment of the neck is not justified. In view of the low incidence of cervical lymph node metastases of merely 3% according to the IRS (Intergroup Rhabdomyosarcoma Study), Rodeberg et al. saw no indication for conducting elective neck dissection (Rodeberg et al.2002). Wurm recommends elective neck dissection in patients with alveolar RMS when surgery on primary tumour is planned (Wurm, 2005). Callender et al. found regional metastasis in 15–38% of their patients respectively and inferred that elective neck dissection offers a benefit (Callender, 1995). Controversy exists also over treating the N+ neck with additional radiotherapy or neck dissection. If nodes are metastatic and complete primary tumour resection is feasible, neck dissection is recommended; while, if the lymph nodes are positive but the primary tumour is unresectable, they should be included in the radiotherapy field.

#### **2.3 Case report**

An 22 year-old female, who had been treated for retinoblastoma at six months of age, presented for evaluation of left temporal swelling. She had underwent enucleation of the bilateral eyeball and 40 Gy irradiation to the left orbit at 1 years of age. Examination revealed a rm mass overlying the left temporal muscle (Figure 1) and left cervical

Fig. 1. A 22-year-old female case of Rabdomyosarcoma, infantile type of the temporal muscle (arrow). She underwent the convination of chemotherapy and radiation 9 years ago, now she was free of disease. (Our experience case)

Neck Dissection of the Head and Neck Sarcoma 129

The incidence of lymph node metastasis of fibrosarcoma was found to be 4.4-5.1 %. It is generally agreed that because of the rarity of lymph node metastasis, elective neck

A 62-year-old male was referred to our hospital for the left neck mass. He had been underwent the removal of the left neck mass and the pathological diagnosis was extraabdominal desmoid. A MRI showed the high intensity mass of the Gd-enhanced T1 weighted image in the Level III area and infiltrate to the surrounding muscles (figure 2). He was underwent the removal of the tumor, encased by the sternocleidmastoid muscle and deep cervical muscles, with preservation of the internal juglar vein and phenic nerve. He

Fig. 2. A 62-year-old male case of desmoid. He underwent the removal encased by the surrounding muscles. After the 3 years operation, now he was free of disease. (Our

Angiosarcomas are a subtype of soft-tissue sarcoma and are aggressive, malignant endothelial-cell tumors of vascular or lymphatic origin (Young et al, 2010). Treatment is challenging in many cases and the prognosis is poor. Angiosarcoma can arise in any softtissue structure or viscera and cutaneous angiosarcomas typically involve the head and neck, particularly the scalp (27%). Angiosarcomas are subdivided into cutaneous angiosarcoma, lymphoedema-associated angiosarcoma, radiation-induced angiosarcoma, rimary-breast angiosarcoma, and soft-tissue angiosarcoma, and most reports include several angiosarcoma subtypes. There is some evidence that tumor behaviour might depend on site of origin, although whether differences between cutaneous, radiation induced, breast, and visceral angiosarcomas are caused by biological differences or differences in clinical

There are no randomised trials and few prospective studies, most published reports of angiosarcoma treatment are retrospective case series. Treatment has been included in

dissection for fibrosarcoma is generally not indicated. (Fong et al, 1993)

**3.2 Lymphatic metastasis and neck dissection** 

**3.3 Case report** 

experience case)

**4.1 Treatment** 

**4. Angiosarcoma** 

presentation and treatment is unclear.

was free of disease after the 3 years.

lymphadenopathy. Biopsy revealed that the tumor was Rabdomyosarcoma, embryonal type. Metastatic work-up was negative including lumber puncture, bone scan ,and chest CT. The patient received treatment with chemotherapy consisting of vincristine 2 mg/m2 , actinomycin D 0.5 mg/m2,and cyclophosphamide 2.2g/m2 (VAC) every 3 weeks for 13 courses. Also she was irradiated 24Gy between 5 courses and 6 courses. She was followed closely after completing chemotherapy. The patient was alive and well with no evidence of disease 80 months after last treatment.

#### **3. Fibrous tumor (Malignant fibrous histiocytoma, Desmoid tumor, and Fibrosarcaoma)**

Since the late 1970s malignant fibrous histiocytoma (MFH) were the most common soft tissue sarcoma of middle and late adulthood. The existence of "malignant fibrous histiocytoma" as a distint entity is now considered controversial and at best is regarded ad a heterogeneous group of tumors without a specific known line of differentiation. Current nomenclature recognizes the entities undifferentiated high grade pleomorphic sarcoma, and myxofibrosarcoma (Montgomery et al. 2009).

Desmoid tumors, or aggressive deep-seated fibromatosis, are part of a rare group of fibrous tissue proliferations which tend to be locally aggressive but have no propensity for metastasis. Desmoid tumors are uncommon and slow-growing tumors with a propensity for recurrence even after complete resection. The natural history of desmoid tumors is not welldefined and poorly understood. Location of tumors can limit therapeutic options and result in significant morbidity with or without surgical resection. While there is no risk of degeneration from fibromatosis to fibrosarcoma, this is a distinct type of soft tissue sarcoma.Unfortunately, there are also no distinct clinical characteristics of fibrosarcoma, and disease is poorly described in the context of all fibroblastic tumors. Classic fibrosarcomas are seen in persons aged 30–55 years. Fibrosarcomas can be part of the spectrum of radiationinduced or radiation-associated sarcomas, representing about 15% of the histologic subtypes. Treatment options are dictated by location, and surgical resection remains the mainstay of curative therapy. Dermatofibrosarcoma protuberans (DFSP) is a specific form of soft tissue sarcoma which is nearly always considered a low-grade sarcoma. These tumors are prone to recurrence, with high local recurrence rates of up to 60% following resection. Occasionally, DFSP will undergo transformation to classic fibrosarcoma (Wong et al, 2008).

#### **3.1 Treatment**

The management of soft tissue sarcomas in the head and neck is primarily surgical. Sarcomas also tend to growth along fascial planes. For these reasons, the surgical resection of sarcomas require a wide resection which respects fascial compartments and includes a generous amount of uninvolved tissue beyond the tumour margins. For most cancer types, a 2.0- to 3.0-cm margin including resection of the underlying fascia is recommended. The critical anatomy of the head and neck limits the capacity to obtain these wide surgical margins. Sarcomas arising in the head and neck have a higher local recurrence rate and a worse disease-specific survival than sarcomas arising at other sites. (Brockstein, 2004)

#### **3.2 Lymphatic metastasis and neck dissection**

The incidence of lymph node metastasis of fibrosarcoma was found to be 4.4-5.1 %. It is generally agreed that because of the rarity of lymph node metastasis, elective neck dissection for fibrosarcoma is generally not indicated. (Fong et al, 1993)

#### **3.3 Case report**

128 Neck Dissection – Clinical Application and Recent Advances

lymphadenopathy. Biopsy revealed that the tumor was Rabdomyosarcoma, embryonal type. Metastatic work-up was negative including lumber puncture, bone scan ,and chest CT. The patient received treatment with chemotherapy consisting of vincristine 2 mg/m2 , actinomycin D 0.5 mg/m2,and cyclophosphamide 2.2g/m2 (VAC) every 3 weeks for 13 courses. Also she was irradiated 24Gy between 5 courses and 6 courses. She was followed closely after completing chemotherapy. The patient was alive and well with no evidence of

**3. Fibrous tumor (Malignant fibrous histiocytoma, Desmoid tumor, and** 

Since the late 1970s malignant fibrous histiocytoma (MFH) were the most common soft tissue sarcoma of middle and late adulthood. The existence of "malignant fibrous histiocytoma" as a distint entity is now considered controversial and at best is regarded ad a heterogeneous group of tumors without a specific known line of differentiation. Current nomenclature recognizes the entities undifferentiated high grade pleomorphic sarcoma, and

Desmoid tumors, or aggressive deep-seated fibromatosis, are part of a rare group of fibrous tissue proliferations which tend to be locally aggressive but have no propensity for metastasis. Desmoid tumors are uncommon and slow-growing tumors with a propensity for recurrence even after complete resection. The natural history of desmoid tumors is not welldefined and poorly understood. Location of tumors can limit therapeutic options and result in significant morbidity with or without surgical resection. While there is no risk of degeneration from fibromatosis to fibrosarcoma, this is a distinct type of soft tissue sarcoma.Unfortunately, there are also no distinct clinical characteristics of fibrosarcoma, and disease is poorly described in the context of all fibroblastic tumors. Classic fibrosarcomas are seen in persons aged 30–55 years. Fibrosarcomas can be part of the spectrum of radiationinduced or radiation-associated sarcomas, representing about 15% of the histologic subtypes. Treatment options are dictated by location, and surgical resection remains the mainstay of curative therapy. Dermatofibrosarcoma protuberans (DFSP) is a specific form of soft tissue sarcoma which is nearly always considered a low-grade sarcoma. These tumors are prone to recurrence, with high local recurrence rates of up to 60% following resection. Occasionally, DFSP will undergo transformation to classic fibrosarcoma (Wong et

The management of soft tissue sarcomas in the head and neck is primarily surgical. Sarcomas also tend to growth along fascial planes. For these reasons, the surgical resection of sarcomas require a wide resection which respects fascial compartments and includes a generous amount of uninvolved tissue beyond the tumour margins. For most cancer types, a 2.0- to 3.0-cm margin including resection of the underlying fascia is recommended. The critical anatomy of the head and neck limits the capacity to obtain these wide surgical margins. Sarcomas arising in the head and neck have a higher local recurrence rate and a worse disease-specific survival than sarcomas arising at other sites.

disease 80 months after last treatment.

myxofibrosarcoma (Montgomery et al. 2009).

**Fibrosarcaoma)** 

al, 2008).

**3.1 Treatment** 

(Brockstein, 2004)

A 62-year-old male was referred to our hospital for the left neck mass. He had been underwent the removal of the left neck mass and the pathological diagnosis was extraabdominal desmoid. A MRI showed the high intensity mass of the Gd-enhanced T1 weighted image in the Level III area and infiltrate to the surrounding muscles (figure 2). He was underwent the removal of the tumor, encased by the sternocleidmastoid muscle and deep cervical muscles, with preservation of the internal juglar vein and phenic nerve. He was free of disease after the 3 years.

Fig. 2. A 62-year-old male case of desmoid. He underwent the removal encased by the surrounding muscles. After the 3 years operation, now he was free of disease. (Our experience case)

#### **4. Angiosarcoma**

Angiosarcomas are a subtype of soft-tissue sarcoma and are aggressive, malignant endothelial-cell tumors of vascular or lymphatic origin (Young et al, 2010). Treatment is challenging in many cases and the prognosis is poor. Angiosarcoma can arise in any softtissue structure or viscera and cutaneous angiosarcomas typically involve the head and neck, particularly the scalp (27%). Angiosarcomas are subdivided into cutaneous angiosarcoma, lymphoedema-associated angiosarcoma, radiation-induced angiosarcoma, rimary-breast angiosarcoma, and soft-tissue angiosarcoma, and most reports include several angiosarcoma subtypes. There is some evidence that tumor behaviour might depend on site of origin, although whether differences between cutaneous, radiation induced, breast, and visceral angiosarcomas are caused by biological differences or differences in clinical presentation and treatment is unclear.

#### **4.1 Treatment**

There are no randomised trials and few prospective studies, most published reports of angiosarcoma treatment are retrospective case series. Treatment has been included in

Neck Dissection of the Head and Neck Sarcoma 131

as well as soft tissues sites may be affected. The peak age of incidence for osteosarcoma outside the head and neck is in the adolescent years. In contrast, HNOS most commonly affects patients in their 30s. Distant metastases have been reported in 10–20% of patients with HNOS, compared with 53–75% of patients with disease arising outside the head and neck. The 5-year, disease-specific survival rate for patients with HNOS has been poor, with most studies reporting survival rates of 23–37%. Management of osteosarcoma outside the head and neck prior to the standardized use of chemotherapy resulted in worse 5-year survival rates of 10–20%.It is widely accepted that wide surgical excision is the mainstay of therapy for patients with HNOS. Adjuvant treatment with radiotherapy and/or chemotherapy also has been administered. For patients with osteosarcoma arising outside the head and neck, surgery also is considered a key component in therapy, but the addition of neoadjuvant and/or adjuvant chemotherapy has improved survival markedly. The Multi-Institution Osteosarcoma Study identified an increase in the 6-year, event-free survival rate from 11% with surgery alone to 61% when chemotherapy was given postoperatively.13 Over the last 2 decades, the standard therapy for patients with non–head and neck osteosarcoma has evolved to routinely include both neoadjuvant and adjuvant chemotherapy. With these regimens, long-term survival rates of 70–80% are being obtained. Whether these same results can be obtained in patients with HNOS is not yet known.

The mainstay of treatment of head and neck osteosarcoma is surgery. Adjuvant postoperative RT is indicated for those with close or positive margins. The role of adjuvant

The AJCC (2010) definitions of TNM showed that any regional lymph node metastasis (N1) classified to Stage IVB. It suggested to the extremely poor prognosis in front of the neck metastasis of the osteosarcoma. In rare case, Extraskeletal osteosarcoma was present ,That is a high-grade malignant sarcomas defined (1) soft tissue origin and no attachment to the bone or periosteum, (2) a uniform sarcomatous pattern (to exclude the possibility of a mixed malignant mesenchymal tumor), with production of osteoid and/or cartilage matrix. We reported a young patient with highly malignant HNOS, which was excised but was

A 17-year-old Japanese man with no history of trauma or irradiation was referred to the hospital with complaints of a swelling in the left side of the neck that had been gradually increasing in size. Physical examination showed a smooth, stony hard, mobile mass measuring 24 x 30 mm in the left submandibular region. A computed tomographic scan (figure 3) showed a calcified mass measuring 23 x 18 mm in size, unattached to the hyoid bone or the mandible. The tumor was mildly adherent to the suprahyoid muscles. It was excised along with surrounding muscle. The surgical margin was free of in the direction of the hyoid bone. Three months after the operation, the patient developed massive local recurrence of the tumor. We removed the tumor encased by the surrounding muscles. Postoperatively, the patient received a course of radiotherapy. However, 4 months after the second operation, he developed left facial nerve palsy and complained of sudden hearing loss on the left side. A CT scan showed

subsequently complicated by local and distant metastases.(Saito et al,2008)

**6.1 Treatment** 

**6.3 Case report** 

chemotherapy is unclear.

**6.2 Lymph node metastasis and neck dissection** 

management guidelines for other soft tissue sarcomas. Radical surgery with complete resection is the primary treatment of choice. Involved margins resection are common because of the invasive and often multifocal nature of angiosarcomas, which confer a worse prognosis. Angiosarcomas have an overall 5 year survival of about 35%. Even with localised disease, the most optimistic survey suggests only 60% of patients survive for more than 5 years, with a median survival of 7 months (Young,2010). However, there are reports of some long-term survivors with metastatic disease, but which prognostic factors are important is unclear.

#### **4.2 Lymphatic metastasis and neck dissection**

The incidence of lymph node metastasis of angiosarcoma was found to be 13.5 % (Fong, 2010). Angiosarcoma is particularly prone to nodal metastasis. Prognosis of such metastasis is poor, but certainly not hopeless. Neck dissection is appropriate treatment for isolated metastasis to regional lymph nodes and might provide long-term survival.

#### **5. Synovial sarcoma**

Synovial sarcoma is a malignant soft-tissue neoplasm uncommonly found in the head and neck. Although synovial sarcoma is usually found near large joints and bursae of the extremities, it rarely arises from synovial membranes. It accounts for approximately 8% of all soft-tissue sarcomas. Only 10% of all synovial sarcomas occur in the head and neck, and these rarely have an obvious association with synovial structures (Amble et al,1997). Synovial sarcoma were most commonly located in the neck (60%); thus, the most common symptoms were a neck mass and neck pain. Ninety percent of Synovial sarcoma have an identifiable translocation between chromosomes 18 and X that results in the fusion of the SYT gene on chromosome 18 and the SSX-1 or SSX-2 gene on chromosome X (Harb et al, 2007).

#### **5.1 Treatment**

As with other soft tissue sarcomas, radical surgery with complete resection is the primary treatment of choice. Unfortunately, up to 50% of Synovial sarcoma recur, usually within 2years. Some 40% metastasis, commonly to lungs and bone and also regional lymph nodes. The best outcome are in childfood patients, in tumors which are <5cm in diameter, have < 10 mitoses/ 10 hpf and no necrosis, and when the tumor is eradicated loccaly. (Christopher et al, 2010)

#### **5.2 Lymph node metastasis and neck dissection**

Although not normally a feature of sarcomas, metastasis to regional lymph nodes occurs in 12.5% of cases of synovial sarcoma in the head and neck and 23% of cases in the extremities (Roth et al, 1975). Neck dissection is appropriate treatment for isolated metastasis to regional lymph nodes and may provide long-term survival.

#### **6. Osteosarcoma**

Osteosarcoma is the most common primary malignancy of bone. These tumors typically originate in the extremities and the pelvis, with only 6–10% of patients presenting with a head and neck primary tumor (Russell et al, 2003). The mandible and maxilla are the predominate locations of head and neck osteosarcoma (HNOS), although extragnathic bone as well as soft tissues sites may be affected. The peak age of incidence for osteosarcoma outside the head and neck is in the adolescent years. In contrast, HNOS most commonly affects patients in their 30s. Distant metastases have been reported in 10–20% of patients with HNOS, compared with 53–75% of patients with disease arising outside the head and neck. The 5-year, disease-specific survival rate for patients with HNOS has been poor, with most studies reporting survival rates of 23–37%. Management of osteosarcoma outside the head and neck prior to the standardized use of chemotherapy resulted in worse 5-year survival rates of 10–20%.It is widely accepted that wide surgical excision is the mainstay of therapy for patients with HNOS. Adjuvant treatment with radiotherapy and/or chemotherapy also has been administered. For patients with osteosarcoma arising outside the head and neck, surgery also is considered a key component in therapy, but the addition of neoadjuvant and/or adjuvant chemotherapy has improved survival markedly. The Multi-Institution Osteosarcoma Study identified an increase in the 6-year, event-free survival rate from 11% with surgery alone to 61% when chemotherapy was given postoperatively.13 Over the last 2 decades, the standard therapy for patients with non–head and neck osteosarcoma has evolved to routinely include both neoadjuvant and adjuvant chemotherapy. With these regimens, long-term survival rates of 70–80% are being obtained. Whether these same results can be obtained in patients with HNOS is not yet known.

#### **6.1 Treatment**

130 Neck Dissection – Clinical Application and Recent Advances

management guidelines for other soft tissue sarcomas. Radical surgery with complete resection is the primary treatment of choice. Involved margins resection are common because of the invasive and often multifocal nature of angiosarcomas, which confer a worse prognosis. Angiosarcomas have an overall 5 year survival of about 35%. Even with localised disease, the most optimistic survey suggests only 60% of patients survive for more than 5 years, with a median survival of 7 months (Young,2010). However, there are reports of some long-term survivors with metastatic disease, but which prognostic factors are important is unclear.

The incidence of lymph node metastasis of angiosarcoma was found to be 13.5 % (Fong, 2010). Angiosarcoma is particularly prone to nodal metastasis. Prognosis of such metastasis is poor, but certainly not hopeless. Neck dissection is appropriate treatment for isolated

Synovial sarcoma is a malignant soft-tissue neoplasm uncommonly found in the head and neck. Although synovial sarcoma is usually found near large joints and bursae of the extremities, it rarely arises from synovial membranes. It accounts for approximately 8% of all soft-tissue sarcomas. Only 10% of all synovial sarcomas occur in the head and neck, and these rarely have an obvious association with synovial structures (Amble et al,1997). Synovial sarcoma were most commonly located in the neck (60%); thus, the most common symptoms were a neck mass and neck pain. Ninety percent of Synovial sarcoma have an identifiable translocation between chromosomes 18 and X that results in the fusion of the SYT gene on

As with other soft tissue sarcomas, radical surgery with complete resection is the primary treatment of choice. Unfortunately, up to 50% of Synovial sarcoma recur, usually within 2years. Some 40% metastasis, commonly to lungs and bone and also regional lymph nodes. The best outcome are in childfood patients, in tumors which are <5cm in diameter, have < 10 mitoses/ 10 hpf and no necrosis, and when the tumor is eradicated loccaly.

Although not normally a feature of sarcomas, metastasis to regional lymph nodes occurs in 12.5% of cases of synovial sarcoma in the head and neck and 23% of cases in the extremities (Roth et al, 1975). Neck dissection is appropriate treatment for isolated metastasis to regional

Osteosarcoma is the most common primary malignancy of bone. These tumors typically originate in the extremities and the pelvis, with only 6–10% of patients presenting with a head and neck primary tumor (Russell et al, 2003). The mandible and maxilla are the predominate locations of head and neck osteosarcoma (HNOS), although extragnathic bone

metastasis to regional lymph nodes and might provide long-term survival.

chromosome 18 and the SSX-1 or SSX-2 gene on chromosome X (Harb et al, 2007).

**4.2 Lymphatic metastasis and neck dissection** 

**5. Synovial sarcoma** 

**5.1 Treatment** 

(Christopher et al, 2010)

**6. Osteosarcoma** 

**5.2 Lymph node metastasis and neck dissection** 

lymph nodes and may provide long-term survival.

The mainstay of treatment of head and neck osteosarcoma is surgery. Adjuvant postoperative RT is indicated for those with close or positive margins. The role of adjuvant chemotherapy is unclear.

#### **6.2 Lymph node metastasis and neck dissection**

The AJCC (2010) definitions of TNM showed that any regional lymph node metastasis (N1) classified to Stage IVB. It suggested to the extremely poor prognosis in front of the neck metastasis of the osteosarcoma. In rare case, Extraskeletal osteosarcoma was present ,That is a high-grade malignant sarcomas defined (1) soft tissue origin and no attachment to the bone or periosteum, (2) a uniform sarcomatous pattern (to exclude the possibility of a mixed malignant mesenchymal tumor), with production of osteoid and/or cartilage matrix. We reported a young patient with highly malignant HNOS, which was excised but was subsequently complicated by local and distant metastases.(Saito et al,2008)

#### **6.3 Case report**

A 17-year-old Japanese man with no history of trauma or irradiation was referred to the hospital with complaints of a swelling in the left side of the neck that had been gradually increasing in size. Physical examination showed a smooth, stony hard, mobile mass measuring 24 x 30 mm in the left submandibular region. A computed tomographic scan (figure 3) showed a calcified mass measuring 23 x 18 mm in size, unattached to the hyoid bone or the mandible. The tumor was mildly adherent to the suprahyoid muscles. It was excised along with surrounding muscle. The surgical margin was free of in the direction of the hyoid bone. Three months after the operation, the patient developed massive local recurrence of the tumor. We removed the tumor encased by the surrounding muscles. Postoperatively, the patient received a course of radiotherapy. However, 4 months after the second operation, he developed left facial nerve palsy and complained of sudden hearing loss on the left side. A CT scan showed

Neck Dissection of the Head and Neck Sarcoma 133

 Head and neck chondrosarcomas are generally slow-growing tumors with an indolent course. Yet, owing to the locally aggressive nature and the propensity to recur if not adequately treated, most studies recommend wide en bloc surgical resection as the mainstay of treatment. Conservative resection of low-grade lesions of the larynx has been proven effective in several reports. Organ preservation may be archived with such an approach,

In the largest series to date representing 151 cases of chondrosarcoma of the head and neck, Weiss and Bennett noted that "metastasis is not common and generally occurs late in the disease, usually after multiple surgical manipulations."(Weiss & Bennett, 1986) Mark et al stated that "only 1/18 (5%) patients recurred in regional lymph nodes without elective neck treatment, further justifying the accepted approach of not electively treating the neck in

A 55-year-old female was referred to us in 2003 because of a 2-years history of hoarseness. She also complained about having dyspnea and had underwent tracheostomy. Direct laryngoscopy revealed a smooth, firm subglottic mass at the subglottis. The CT scan showed a mass that had eroded almost half of the left cricoid cartilage (Figure 4), and the biopsy revealed chondroma. An excision of the tumor and 4/5 cricoid cartilage was resected. A tracheofissure was done at the end of the operation to put a stent T tube in place. Histopathologic examination of the surgical specimen confirmed a grade I chondrosarcoma. 27 months later, direct laryngoscopy was done with no evidence of recurrence. Afterall, we reconstructed the anterior wall of trachea by the costal cartilage. Up until now, the patient has been followed up regularly. In May 2011, 8 years after surgery, she was in perfect health

Fig. 4. A case of laryngeal chondrosarcoma of 40-year-old female. She was underwent operation with laryngeal preservation 9 years ago. Now she was free of disease with

which has important functional outcome implications (Hong et al, 2009).

**7.2 Lymph node metastasis and neck dissection** 

head and neck sarcomas."(Mark et al, 1993)

except for a paralysis of the left vocal cord.

laryngeal function. (Our experienced case)

**7.1 Treatment** 

**7.3 Case report** 

tumor recurrence in the temporal bone, occupying the middle cranial fossa. The patient and his family selected chemotherapy instead of surgery, but 1month after the start of chemotherapy consisting of Cisplatin 125mg/body and Pirarubicin 60mg/body, the patient developed intracranial hemorrhage and died of hydrocephalus.

Fig. 3. A case of extraskeletal osteosarcoma of 17-year-old male. (Our experienced case)

#### **7. Chondrosarcoma**

Chondrosarcoma is a malignant tumor in which the tumor cells form chondroid (cartilage) but not osteoid. Although chondrosarcoma most commonly arises from either cartilaginous structures or bone derived from chondroid precursors, chondrosarcoma may also arise in areas where cartilage is not normally found. Skull bones were the predominant tissue (59.7%) arose chondrosarcoma. Among these cases were 7.7% originating in the mandible. laryngotracheal cases were about 23.4% in all cases. The other sites were oral cavity, pharynx, and orbit (Koch et al, 2000).

These tumors developing in soft tissue presumably arise from cartilaginous differentiation of primitive mesenchymal cells. Chondrosarcomas develop more commonly in bone than cartilage. However, a sufficiently large number develop from laryngeal cartilage, so that chondrosarcoma is the most common sarcoma developing in the larynx.

It is noteworthy that more than 90% of laryngotracheal chondrosarcoma cases were low grade and occurred in older patients (>50 years). These associations suggest that laryngotracheal tumors represent a form of chondrosarcoma that should be distinguished from cases arising in osseous sites of the head and neck that are more commonly characterized by a younger age and a higher grade. The recognized difficulty in discriminating between the benign laryngeal chondromatous lesions and low-grade chondrosarcomas confounds the interpretation of tumor behavior at this site. It is possible that the large number of low-grade chondrosarcomas identified within the cartilaginous laryngotracheal site may reflect the inclusion of some benign cases erroneously diagnosed as cancer. Despite ongoing study, a widely accepted approach to analysis of cytogenic or proliferative characteristics has yet to surpass grading as an independent predictor of the biologic behavior of chondrosarcoma (Koch et al,2000).

#### **7.1 Treatment**

132 Neck Dissection – Clinical Application and Recent Advances

tumor recurrence in the temporal bone, occupying the middle cranial fossa. The patient and his family selected chemotherapy instead of surgery, but 1month after the start of chemotherapy consisting of Cisplatin 125mg/body and Pirarubicin 60mg/body, the patient

Fig. 3. A case of extraskeletal osteosarcoma of 17-year-old male. (Our experienced case)

Chondrosarcoma is a malignant tumor in which the tumor cells form chondroid (cartilage) but not osteoid. Although chondrosarcoma most commonly arises from either cartilaginous structures or bone derived from chondroid precursors, chondrosarcoma may also arise in areas where cartilage is not normally found. Skull bones were the predominant tissue (59.7%) arose chondrosarcoma. Among these cases were 7.7% originating in the mandible. laryngotracheal cases were about 23.4% in all cases. The other sites were oral cavity,

These tumors developing in soft tissue presumably arise from cartilaginous differentiation of primitive mesenchymal cells. Chondrosarcomas develop more commonly in bone than cartilage. However, a sufficiently large number develop from laryngeal cartilage, so that

It is noteworthy that more than 90% of laryngotracheal chondrosarcoma cases were low grade and occurred in older patients (>50 years). These associations suggest that laryngotracheal tumors represent a form of chondrosarcoma that should be distinguished from cases arising in osseous sites of the head and neck that are more commonly characterized by a younger age and a higher grade. The recognized difficulty in discriminating between the benign laryngeal chondromatous lesions and low-grade chondrosarcomas confounds the interpretation of tumor behavior at this site. It is possible that the large number of low-grade chondrosarcomas identified within the cartilaginous laryngotracheal site may reflect the inclusion of some benign cases erroneously diagnosed as cancer. Despite ongoing study, a widely accepted approach to analysis of cytogenic or proliferative characteristics has yet to surpass grading as an independent predictor of the

chondrosarcoma is the most common sarcoma developing in the larynx.

biologic behavior of chondrosarcoma (Koch et al,2000).

developed intracranial hemorrhage and died of hydrocephalus.

**7. Chondrosarcoma** 

pharynx, and orbit (Koch et al, 2000).

 Head and neck chondrosarcomas are generally slow-growing tumors with an indolent course. Yet, owing to the locally aggressive nature and the propensity to recur if not adequately treated, most studies recommend wide en bloc surgical resection as the mainstay of treatment. Conservative resection of low-grade lesions of the larynx has been proven effective in several reports. Organ preservation may be archived with such an approach, which has important functional outcome implications (Hong et al, 2009).

#### **7.2 Lymph node metastasis and neck dissection**

In the largest series to date representing 151 cases of chondrosarcoma of the head and neck, Weiss and Bennett noted that "metastasis is not common and generally occurs late in the disease, usually after multiple surgical manipulations."(Weiss & Bennett, 1986) Mark et al stated that "only 1/18 (5%) patients recurred in regional lymph nodes without elective neck treatment, further justifying the accepted approach of not electively treating the neck in head and neck sarcomas."(Mark et al, 1993)

#### **7.3 Case report**

A 55-year-old female was referred to us in 2003 because of a 2-years history of hoarseness. She also complained about having dyspnea and had underwent tracheostomy. Direct laryngoscopy revealed a smooth, firm subglottic mass at the subglottis. The CT scan showed a mass that had eroded almost half of the left cricoid cartilage (Figure 4), and the biopsy revealed chondroma. An excision of the tumor and 4/5 cricoid cartilage was resected. A tracheofissure was done at the end of the operation to put a stent T tube in place. Histopathologic examination of the surgical specimen confirmed a grade I chondrosarcoma. 27 months later, direct laryngoscopy was done with no evidence of recurrence. Afterall, we reconstructed the anterior wall of trachea by the costal cartilage. Up until now, the patient has been followed up regularly. In May 2011, 8 years after surgery, she was in perfect health except for a paralysis of the left vocal cord.

Fig. 4. A case of laryngeal chondrosarcoma of 40-year-old female. She was underwent operation with laryngeal preservation 9 years ago. Now she was free of disease with laryngeal function. (Our experienced case)

Neck Dissection of the Head and Neck Sarcoma 135

Allam A, El-Husseiny G, Khafaga Y, Kandil A, Gray A, Ezzat A, Schultz H. (1999)Ewing's

Amble FR, Olsen KD, Nascimento AG, Foote RL(1992). Head and neck synovial cell

Brockstein B (2004). Management of sarcomas of the head and neck. *Curr Oncol Rep.*

Callender TA, Weber RS, Janjan N, Benjamin R, Zaher M, Wolf P, el-Naggar A(1995).

Costa MJ, Campman SC, Davis RL, Howell LP (1996). Fine-needle aspiration cytology of

Christopher D.M.Fletcher, K.Krishnan Unni, Fredrik Mertens (2010).*World Heath* 

Christopher D.M.Fletcher, K.Krishnan Unni, Fredrik Mertens (2010).*World Heath* 

de Bree R, van der Waal I, de Bree E, Leemans CR(2010). Management of adult soft tissue

Fong Y, Coit DG, Woodruff JM, Brennan MF(1993). Lymph node metastasis from soft tissue

Fyrmpas G, Wurm J, Athanassiadou F, Papageorgiou T, Beck JD, Iro H, Constantinidis

Gradoni P, Giordano D, Oretti G, Fantoni M, Ferri T(2010). The role of surgery in children

Harb WJ, Luna MA, Patel SR, Ballo MT, Roberts DB, Sturgis EM(2007). Survival in patients

Hong P, Taylor SM, Trites JR, Bullock M, Nasser JG, Hart RD (2009). Chondrosarcoma of the

Jones C, Liu K, Hirschowitz S, Klipfel N, Layfield LJ (2002). Concordance of histopathologic

Koch BB, Karnell LH, Hoffman HT, Apostolakis LW, Robinson RA, Zhen W, Menck

Ludwig JA(2008). Ewing sarcoma: historical perspectives, current state-of-the-art, and

*Bone.* 9 Tumours of uncertain differentiation pp.200-204,IARC

sarcomas of the head and neck. *Oral Oncol.* 46(11),Nov,pp.786-90

sarcoma. *Otolaryngol Head Neck Surg.* 107(5),Nov,pp.631-7.

*Otolaryngol Head Neck Surg.* 112(2),Feb,pp.252-257.

*Bone.* 6 Skeletal muscle tumours pp.127-134,IARC

patients. *Ann Surg.* 217(1),Apr,pp.72-77.

and extension. *Head Neck.* 29(8),Aug,pp.731-40.

Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases. *Sarcoma.* 

Rhabdomyosarcoma of the nose and paranasal sinuses in adults and children.

sarcoma: retrospective review of diagnostic utility and specificity. *Diagn Cytopathol.* 

*Organization Classification of Tumours. Pathology & Genetics Tumours of Soft Tissue and* 

*Organization Classification of Tumours. Pathology & Genetics Tumours of Soft Tissue and* 

sarcoma in adults. Analysis of data from a prospective database of 1772 sarcoma

J.(2009) Management of paediatric sinonasal rhabdomyosarcoma. *J Laryngol Otol.* 

with head and neck rhabdomyosarcoma and Ewing's sarcoma. *Surg Oncol.*

with synovial sarcoma of the head and neck: association with tumor location, size,

head and neck: report of 11 cases and literature review. *J Otolaryngol Head Neck* 

and cytologic grading in musculoskeletal sarcomas: can grades obtained from analysis of the fine-needle aspirates serve as the basis for therapeutic decisions?

HR(2000). National cancer database report on chondrosarcoma of the head and

opportunities for targeted therapy in the future. *Curr Opin Oncol.* 20(4),Jul,pp.412-8.

**10. References** 

3(1):pp.11-5.

6(4),Jul,pp321-327

15(1),Jul,pp23-32.

123(9),Sep,pp.990-6.

19(4),Dec,pp.e103-9.

*Surg.* 38(2),Apr,pp.279-85

*Cancer.* 96(2),Apr,pp83-91.

neck. *Head Neck.* 22(4),Jul,pp.408-25.

#### **8. Ewing's sarcoma**

Ewing's sarcoma arising in the head and neck region is extremely rare, comprising 1-4% of all cases of Ewing's sarcoma. Most authors claim a better prognosis for Ewing's sarcoma of the head and neck region as compared to that arising in other anatomic locations. The most commonly affected bones in the head and neck region are the skull, the mandible and the maxilla. There has also been case reports of localized Ewing's sarcoma affecting the orbital roof, the retropharynx and the nasal cavity (Allam et al, 1999).

#### **8.1 Treatment**

Surgery after chemotherapy is the current standard of cure. Despite the lack of randomized trials comparing different local treatment modalities in Ewing' Sarcoma, wide surgical resection is considered the treatment of choice. It reduces the local relapse rate, improves the overall survival and may avoid radiotherapy. Ludwig affirms that chemotherapy alone is an ineffective measure in achieving local control of disease (Ludwig, 2008). Thus, it is commonly accepted that an attempt at complete tumour resection should always be made. Nevertheless chemoradiation may be a reasonable alternative to surgery for treatment of tumours that are resectable only with an unacceptable degree of mutilation.

#### **8.2 Lymph node metastasis and neck dissection**

Lymphnodes metastasis from Ewing's sarcoma are very rare. The nodal status included as a prognostic indicator in the American Joint Committee on Cancer (AJCC) staging system has even been considered irrelevant by some authors. Considering this, it can be argued that elective neck dissection is not indicated. Nevertheless, as best as we could determine, this argument is not discussed in the literature.

#### **9. Conclusion**

Head and neck sarcomas are rare tumors and represent a heterogeneous group of tumours of different histological variants. Subtyping of sarcomas is increasingly important because of the development of biologic response modifiers. Although traditional morphological assessment is the foundation of clinical decision making, immunohistochemistry and molecular biology are useful for diagnosis, prognosis and identification of possible targets for molecular therapy. The management of sarcomas in the head and neck is primarily surgical. Since, in head and neck sarcomas it is difficult to obtain wide margins during surgical treatment, because of anatomic constraints, most patients with locally resectable tumours undergo post-operative irradiation. Modern reconstructive surgery makes more extensively resection possible and may improve local tumour control, while providing acceptable cosmetic and functional results. Adjuvant systemic chemotherapy seems to improve outcome, but its benefit must be weighted against associated toxicities. Survival of patients with head and neck soft tissue sarcoma varies from 50 to 80% and depends on prognostic factors as tumour grade, margin status and tumour size. With further insight into the biology of soft tissue sarcoma and the combination of new treatment options with modern imaging techniques, we will most certainly be able to improve clinical outcome in patients with soft tissue sarcoma in the upcoming years.

#### **10. References**

134 Neck Dissection – Clinical Application and Recent Advances

Ewing's sarcoma arising in the head and neck region is extremely rare, comprising 1-4% of all cases of Ewing's sarcoma. Most authors claim a better prognosis for Ewing's sarcoma of the head and neck region as compared to that arising in other anatomic locations. The most commonly affected bones in the head and neck region are the skull, the mandible and the maxilla. There has also been case reports of localized Ewing's sarcoma affecting the orbital

Surgery after chemotherapy is the current standard of cure. Despite the lack of randomized trials comparing different local treatment modalities in Ewing' Sarcoma, wide surgical resection is considered the treatment of choice. It reduces the local relapse rate, improves the overall survival and may avoid radiotherapy. Ludwig affirms that chemotherapy alone is an ineffective measure in achieving local control of disease (Ludwig, 2008). Thus, it is commonly accepted that an attempt at complete tumour resection should always be made. Nevertheless chemoradiation may be a reasonable alternative to surgery for treatment of

Lymphnodes metastasis from Ewing's sarcoma are very rare. The nodal status included as a prognostic indicator in the American Joint Committee on Cancer (AJCC) staging system has even been considered irrelevant by some authors. Considering this, it can be argued that elective neck dissection is not indicated. Nevertheless, as best as we could determine, this

Head and neck sarcomas are rare tumors and represent a heterogeneous group of tumours of different histological variants. Subtyping of sarcomas is increasingly important because of the development of biologic response modifiers. Although traditional morphological assessment is the foundation of clinical decision making, immunohistochemistry and molecular biology are useful for diagnosis, prognosis and identification of possible targets for molecular therapy. The management of sarcomas in the head and neck is primarily surgical. Since, in head and neck sarcomas it is difficult to obtain wide margins during surgical treatment, because of anatomic constraints, most patients with locally resectable tumours undergo post-operative irradiation. Modern reconstructive surgery makes more extensively resection possible and may improve local tumour control, while providing acceptable cosmetic and functional results. Adjuvant systemic chemotherapy seems to improve outcome, but its benefit must be weighted against associated toxicities. Survival of patients with head and neck soft tissue sarcoma varies from 50 to 80% and depends on prognostic factors as tumour grade, margin status and tumour size. With further insight into the biology of soft tissue sarcoma and the combination of new treatment options with modern imaging techniques, we will most certainly be able to improve clinical outcome in

tumours that are resectable only with an unacceptable degree of mutilation.

roof, the retropharynx and the nasal cavity (Allam et al, 1999).

**8.2 Lymph node metastasis and neck dissection** 

patients with soft tissue sarcoma in the upcoming years.

argument is not discussed in the literature.

**8. Ewing's sarcoma** 

**8.1 Treatment** 

**9. Conclusion** 


**10** 

*Turkey* 

**Oncocytic Neoplasms** 

*and Neck Surgery, Central Campus,Kutahya* 

*Head and Neck Surgery, Golkoy, Bolu,* 

Fatih Oghan1, Tayfun Apuhan2 and Ali Guvey1

**Rare Malignant Tumors of the Parotid Glands:** 

*1Dumlupinar University, Faculty of Medicine, Department of Otorhinolaryngology Head* 

*2Abant Izzet Baysal University, Faculty of Medicine, Department of Otorhinolaryngology* 

Salivary gland neoplasms are a rare group of tumors; the annual incidence rate is 1 in 100,000 individual, comprising about 3% of all head and neck neoplasms [4]. The mean age of patients with salivary gland tumors is 45 years, peaking in the sixth and seventh decades of life. Benign salivary gland tumors occur more frequently in females, while malignant tumors are slightly more frequent in males. The parotid gland is the most frequent site about 70% of cases. About 80% of parotid tumors are benign and 64 to 80% of all primary salivary gland epithelial tumors involve the parotid gland, mostly located in the superficial

Oncocytic neoplasms comprise a group of rare tumours of the parotid glands, and their incidence represents approximately 1% of parotid neoplasms [1]. Histologically they are classified according to the new World Health Organization classification in three distinct types, namely oncocytosis, oncocytoma and oncocytic carcinoma [2]. Oncocytomas usually occur in the elderly and affect the parotid glands in 80% [3]. Pathologically, oncocytoma is described as a well circumscribed mass, composed of layers of oncocytes (small round nucleus, micro-granular, eosinophilic cytoplasm). Oncocytes are large, granular, eosinophilic epithelial cells mainly found in glandular tissue, including that of the salivary glands and thyroid. Oncocytomas that originate from oncocytes are very rare neoplasms

Oncocytic carcinomas are even more uncommon; they represent 11% of all oncocytic salivary gland neoplasms, 0.5% of all epithelial salivary gland malignancies and 0.18% of all epithelial salivary gland tumors [5, 10]. The terms oncocytic carcinoma, oncocytic adenocarcinoma, malignant oncocytoma and malignant oxyphilic adenoma are synonymous [6]. Besides these defined neoplasms, there are other entities that are not well identified oncocytic changes of the parotid gland, such as oncocytic metaplasia, diffuse oncocytosis, nodular oncocytosis and multifocal nodular oncocytic hyperplasia and also oncocytic

While the diagnosis of these lesions is usually straightforward, the histologic distinction between nodular oncocytosis and oncocytoma is admittedly rather arbitrary in certain cases.

metaplasia within other salivary gland tumors such as the Warthin's tumor [7].

that account for less than 1% of all salivary gland tumors [4].

**1. Introduction** 

lobe [5].

