Intradural Extramedullary Spinal Tumors

*Saleh Rasras and Arash Kiani*

## **Abstract**

Intradural extramedullary (IDEM) spinal tumors are common pathologies, and despite their name, they can extend beyond dural confinements. IDEMs can have both sporadic and syndromic patterns, and various genetic abnormalities are believed to be responsible for these mainly benign pathologies. Meningiomas, nerve sheath tumors (NST), and ependymomas are the three most common subtypes, and due to their pathologically benign nature, surgical total resection plays the most important role in their management. These tumors have always been challenging entities to neurosurgeons, and many surgical techniques have been described in order to achieve gross total resection, and these techniques have continued to evolve over time. Adjuvant therapies such as radiotherapy or radiosurgery are usually considered when total resection is not possible or sometimes in syndromic patients in order to avoid multiple surgical procedures in a short period of time.

**Keywords:** intradural extramedullary (IDEM), spinal cord, tumor, nerve sheath tumors (NST), meningioma, ependymoma

## **1. Introduction**

A wide variety of tumors can affect spinal column and cord causing functional or neurological impairment. Axial skeleton tumors can be either primary or secondary with metastatic lesions being the most common and are classified as secondary malignant tumors. On the other hand, primary tumors of the axial skeletons are the ones arising from vertebral bony structures and can also be benign or malignant.

The skeletal system is one the most common sites for metastasis with spinal part being the most frequent site [1] due to its cancellous structure and extensive arterial and venous supplies [2]. Cancers with tendency to affect the spinal column are in descending order: prostate, breast, kidney, lung, and thyroid [3] (**Figure 1**).

Benign tumors of the spinal column can be diagnosed in both children and adults; in children they could be similar to the tumors of other skeletal areas like giant cell tumors (GCT) or osteoblastomas. Regarding the autopsy studies, vertebral hemangiomas are the most common benign primary lesions of the spinal column in adults and could be seen in up to 20% of the population. Other common benign primary tumors are aneurysmal bone cyst, osteoblastoma, osteoid osteoma, GCT, osteochondroma, and enchondroma (**Figure 1**) [4].

Malignant primary spinal column tumors tend to occur in older patients than primary ones, and the most commonly occurring tumors are multiple myeloma and plasmacytoma, chordoma, and osteosarcoma in order of frequency (**Figure 1**).

**Figure 1.** *Spinal column tumors.*

**45**

*Intradural Extramedullary Spinal Tumors DOI: http://dx.doi.org/10.5772/intechopen.85360*

at the nerve root entry zone areas.

epidermoid cysts (**Figure 2**).

ependymomas.

shape pattern.

schwannomas.

pattern (Antoni B) [11].

50–70 years of age [8].

ment of intradural extramedullary tumors.

**2. Epidemiology and histology**

subependymomas, and in very rare cases lymphomas.

Tumors that arise within the dural sac are termed as intradural tumors and can be within the substance of the spinal cord (intramedullary) or outside of it (extramedullary); however, a small portion of tumors can be both intra- and extramedullary and usually are seen at the conus medullaris transition site to filum terminale or

Intramedullary tumors are usually benign but can also be malignant, and the most common pathologies are astrocytoma, ependymoma, and hemangioblastoma (**Figure 2**); other less common tumors are gangliogliomas, oligodendrogliomas,

Intradural extramedullary (IDEM) tumors consist almost 70% of all intradural lesions [5], and the most frequent types are nerve sheath tumors, meningiomas, and myxopapillary ependymomas. Other less common tumors include hemangiopericytomas, lipomas, paragangliomas, and inclusion cysts such as dermoid and

In this chapter we focus on fundamentals of assessment, diagnosis, and treat-

IDEM tumors are mostly consisted of meningiomas, nerve sheath tumors (schwannomas and neurofibromas), and at the filum terminale myxopapillary

Meningiomas are the most frequent intradural tumors and usually happen at the thoracic region. Psammomatous subtype is the most common histologic subtype, and they resemble the intracranial ones in which numerous psammoma bodies can be observed [6, 7]. Meningiomas have female preponderance with female to male ratio of 3–4:1 and tend to affect the elder population of

Nerve sheath tumors affect both sexes equally with the peak incidence in the fourth and fifth decade of life. Schwannomas are the far more common subtype in this category and usually happen sporadically but also can be seen in neurofibromatosis type 2 [9]. Spinal NSTs arise from ventral or dorsal nerve rootlets with the dorsal nerve rootlet being more common. These tumors can be purely extradural especially at the cervical regions or purely intradural; they also can have both intra- and extradural components and present in dumbbell

The transition zone of the myelin-producing cells from oligodendrocytes to Schwann cell is believed to be where schwannomas arise from a nonfunctional nerve fascicle, and as they grow, these well-capsulated lesions can cause compression on adjacent functional fascicles [10]. Schwannomas can be seen in a compact cellular pattern with palisading verocay bodies (Antoni A) or in a less cellular

Neurofibromas primarily are seen in patients with neurofibromatosis type 2 but can also happen sporadically. Unlike schwannomas these tumors can involve multiple nerve fascicles and expand the whole nerve which makes it sometimes impossible to totally resect the tumor without sacrificing the nerve of origin. The presence of axons in gross pathology can help in distinguishing these lesions from

Filum terminale ependymomas are well-capsulated tumors with slight male preponderance with peak incidence at 36 years of age [12]. Histologic smears reveal well-differentiated radially arranged cuboidal or columnar cells around vascular-

ized myxoid cores with a myxopapillary appearance.

**Figure 2.** *Intradural spinal cord tumors.*

*Intradural Extramedullary Spinal Tumors DOI: http://dx.doi.org/10.5772/intechopen.85360*

*Brain and Spinal Tumors - Primary and Secondary*

**44**

**Figure 2.**

**Figure 1.**

*Spinal column tumors.*

*Intradural spinal cord tumors.*

Tumors that arise within the dural sac are termed as intradural tumors and can be within the substance of the spinal cord (intramedullary) or outside of it (extramedullary); however, a small portion of tumors can be both intra- and extramedullary and usually are seen at the conus medullaris transition site to filum terminale or at the nerve root entry zone areas.

Intramedullary tumors are usually benign but can also be malignant, and the most common pathologies are astrocytoma, ependymoma, and hemangioblastoma (**Figure 2**); other less common tumors are gangliogliomas, oligodendrogliomas, subependymomas, and in very rare cases lymphomas.

Intradural extramedullary (IDEM) tumors consist almost 70% of all intradural lesions [5], and the most frequent types are nerve sheath tumors, meningiomas, and myxopapillary ependymomas. Other less common tumors include hemangiopericytomas, lipomas, paragangliomas, and inclusion cysts such as dermoid and epidermoid cysts (**Figure 2**).

In this chapter we focus on fundamentals of assessment, diagnosis, and treatment of intradural extramedullary tumors.

## **2. Epidemiology and histology**

IDEM tumors are mostly consisted of meningiomas, nerve sheath tumors (schwannomas and neurofibromas), and at the filum terminale myxopapillary ependymomas.

Meningiomas are the most frequent intradural tumors and usually happen at the thoracic region. Psammomatous subtype is the most common histologic subtype, and they resemble the intracranial ones in which numerous psammoma bodies can be observed [6, 7]. Meningiomas have female preponderance with female to male ratio of 3–4:1 and tend to affect the elder population of 50–70 years of age [8].

Nerve sheath tumors affect both sexes equally with the peak incidence in the fourth and fifth decade of life. Schwannomas are the far more common subtype in this category and usually happen sporadically but also can be seen in neurofibromatosis type 2 [9]. Spinal NSTs arise from ventral or dorsal nerve rootlets with the dorsal nerve rootlet being more common. These tumors can be purely extradural especially at the cervical regions or purely intradural; they also can have both intra- and extradural components and present in dumbbell shape pattern.

The transition zone of the myelin-producing cells from oligodendrocytes to Schwann cell is believed to be where schwannomas arise from a nonfunctional nerve fascicle, and as they grow, these well-capsulated lesions can cause compression on adjacent functional fascicles [10]. Schwannomas can be seen in a compact cellular pattern with palisading verocay bodies (Antoni A) or in a less cellular pattern (Antoni B) [11].

Neurofibromas primarily are seen in patients with neurofibromatosis type 2 but can also happen sporadically. Unlike schwannomas these tumors can involve multiple nerve fascicles and expand the whole nerve which makes it sometimes impossible to totally resect the tumor without sacrificing the nerve of origin. The presence of axons in gross pathology can help in distinguishing these lesions from schwannomas.

Filum terminale ependymomas are well-capsulated tumors with slight male preponderance with peak incidence at 36 years of age [12]. Histologic smears reveal well-differentiated radially arranged cuboidal or columnar cells around vascularized myxoid cores with a myxopapillary appearance.

## **3. Genetic considerations**

Genetic syndromes such as neurofibromatosis can be associated with IDEMs. Spinal neurofibromas can be associated with NF1 and NF2 in which NF1 is far more common than NF2.

Neurofibromatosis type 1 (NF1) caused by a mutation in the NF1 gene on chromosome 17 that codes neurofibromin is an autosomal dominant inherited syndrome that can be associated with multiple spinal neurofibromas.

Schwannomas, neurofibromas, and meningiomas are associated with NF2 which is inherited as an autosomal dominant syndrome and caused by mutation at chromosome 22 (NF2 gene) that codes merlin protein which is the responsible etiology [13]. Schwannomatosis a syndrome which is characterized by multiple schwannomas without defining other features of NF1 or NF2 is also another syndrome that may cause spinal schwannomas.

Spinal irradiation and NF2 are two main predisposing factors that cause spinal meningiomas. Intramedullary ependymomas are associated with NF2, but myxopapillary ependymoma is believed to be a distinct entity and is not related to NF2.

#### **4. Sign and symptoms**

IDEM tumors are usually benign slow-growing tumors, and there can be a long period of time between the initiation of symptoms and the diagnosis. Axial back pain can be present for a long time before diagnosis and can be the only symptom. Radicular pain is another symptom especially in patients with NSTs. Spinal cord compression can cause myelopathy or cauda equina syndrome.

Syndromic patients may reveal symptoms at younger age with more rapid progression of functional or neural impairment.

#### **5. Imaging**

Plain X-ray is not usually indicated in evaluation of patients with IDEM tumors, but due to the slow-growing nature of these tumors, reactive bony responses such as foraminal widening, vertebral body scalloping, laminar thinning, and increased inter-pedicular space can be seen.

Computed tomographic studies are quite helpful regarding the evaluation of bony structures, spinal stability, and tumoral calcification and are also helpful in surgical planning.

Magnetic resonance imaging is the modality of choice for the diagnosis of these lesions and delineating their relative anatomy regarding the spinal cord and nerve rootlets.

Schwannomas and neurofibromas have decreased or equal signals in T1W imaging and increased signal in T2W imaging, and they show avid heterogeneous or homogeneous enhancement in contrast studies (**Figure 3**).

Meningiomas have more homogeneous imaging patterns than NSTs and show equal to decreased signals in T1WI and equal to slightly increased signals in T2WI; they show more homogeneous contrast enhancement, and dural enhancement (dural tail) can also be observed (**Figure 4**).

Myxopapillary ependymomas usually represent themselves as isointense lesions in T1W imaging, but the mucinous component can show hypersignality in T1WI. Ependymomas are usually hypersignal in T2W imaging studies and

**47**

**Figure 4.**

*thoracic meningioma.*

**Figure 3.**

are enhanced in contrast studies. Myxopapillary ependymomas are most prone to hemorrhage, and when present, MR images show heterogeneous signals and

*Sagittal T2W (A), T1 non-contrast, (B) and contrast-enhanced (C) images of a patient with ventral thoracic meningioma. Sagittal T1W (D), T2W (E), and (F) T1 contrast-enhanced images of a patient with dorsal* 

*T1W non-contrast-enhanced (A), T1W contrast-enhanced (B), and T2W (C) axial images of a thoracic schwannoma showing cord compression and massive retroperitoneal and paraspinal component. Midsagittal T1W and T2W MR images of a patient with thoracic schwannoma (D). Axial T2W MR image of a syndromic* 

heterogeneous enhancement pattern (**Figure 5**).

*Intradural Extramedullary Spinal Tumors DOI: http://dx.doi.org/10.5772/intechopen.85360*

*patient with NF1 showing bilateral spinal neurofibroma (E).*

#### **Figure 3.**

*Brain and Spinal Tumors - Primary and Secondary*

that can be associated with multiple spinal neurofibromas.

compression can cause myelopathy or cauda equina syndrome.

homogeneous enhancement in contrast studies (**Figure 3**).

(dural tail) can also be observed (**Figure 4**).

progression of functional or neural impairment.

inter-pedicular space can be seen.

Genetic syndromes such as neurofibromatosis can be associated with IDEMs. Spinal neurofibromas can be associated with NF1 and NF2 in which NF1 is far more

Neurofibromatosis type 1 (NF1) caused by a mutation in the NF1 gene on chromosome 17 that codes neurofibromin is an autosomal dominant inherited syndrome

Schwannomas, neurofibromas, and meningiomas are associated with NF2 which is inherited as an autosomal dominant syndrome and caused by mutation at chromosome 22 (NF2 gene) that codes merlin protein which is the responsible etiology [13]. Schwannomatosis a syndrome which is characterized by multiple schwannomas without defining other features of NF1 or NF2 is also another syndrome that

Spinal irradiation and NF2 are two main predisposing factors that cause spinal meningiomas. Intramedullary ependymomas are associated with NF2, but myxopapillary ependymoma is believed to be a distinct entity and is not related

IDEM tumors are usually benign slow-growing tumors, and there can be a long period of time between the initiation of symptoms and the diagnosis. Axial back pain can be present for a long time before diagnosis and can be the only symptom. Radicular pain is another symptom especially in patients with NSTs. Spinal cord

Syndromic patients may reveal symptoms at younger age with more rapid

Plain X-ray is not usually indicated in evaluation of patients with IDEM tumors, but due to the slow-growing nature of these tumors, reactive bony responses such as foraminal widening, vertebral body scalloping, laminar thinning, and increased

Computed tomographic studies are quite helpful regarding the evaluation of bony structures, spinal stability, and tumoral calcification and are also helpful in

Magnetic resonance imaging is the modality of choice for the diagnosis of these lesions and delineating their relative anatomy regarding the spinal cord and nerve

Schwannomas and neurofibromas have decreased or equal signals in T1W imaging and increased signal in T2W imaging, and they show avid heterogeneous or

Meningiomas have more homogeneous imaging patterns than NSTs and show equal to decreased signals in T1WI and equal to slightly increased signals in T2WI; they show more homogeneous contrast enhancement, and dural enhancement

Myxopapillary ependymomas usually represent themselves as isointense lesions in T1W imaging, but the mucinous component can show hypersignality in T1WI. Ependymomas are usually hypersignal in T2W imaging studies and

**3. Genetic considerations**

may cause spinal schwannomas.

**4. Sign and symptoms**

common than NF2.

to NF2.

**5. Imaging**

surgical planning.

rootlets.

**46**

*T1W non-contrast-enhanced (A), T1W contrast-enhanced (B), and T2W (C) axial images of a thoracic schwannoma showing cord compression and massive retroperitoneal and paraspinal component. Midsagittal T1W and T2W MR images of a patient with thoracic schwannoma (D). Axial T2W MR image of a syndromic patient with NF1 showing bilateral spinal neurofibroma (E).*

#### **Figure 4.**

*Sagittal T2W (A), T1 non-contrast, (B) and contrast-enhanced (C) images of a patient with ventral thoracic meningioma. Sagittal T1W (D), T2W (E), and (F) T1 contrast-enhanced images of a patient with dorsal thoracic meningioma.*

are enhanced in contrast studies. Myxopapillary ependymomas are most prone to hemorrhage, and when present, MR images show heterogeneous signals and heterogeneous enhancement pattern (**Figure 5**).

**Figure 5.** *Sagittal T2W (A), T1 non-contrast, (B) and contrast-enhanced (C) images of a patient with filum terminale ependymoma.*

## **6. Indications for surgery and surgical routes**

Surgical intervention is required in almost all symptomatic patients, but in syndromic patients with mild symptoms, due to higher chance of regrowth and multiple lesions, surgery might not be performed in order to avoid multiple surgeries in a short period of time.

All patients with progressive neural or functional impairment and those with rapid tumor growth in serial MR studies should undergo surgery.

Asymptomatic patients can be followed by serial clinical and radiological examinations, and surgery is not advised for diagnostic purposes only; the only exception would be myxopapillary ependymoma in which asymptomatic patients may be advised to undergo surgical evacuation for CSF seeding prevention [14, 15].

Most of IDEM tumors can be approached via simple posterior standard laminectomy, though the tumor location in the spinal axis and its relation to the spinal cord are the major factors determining the surgical route.

Cervical lesions can be addressed via both anterior and posterior approaches. Tumors located posteriorly, laterally, and ventrolaterally can be approached by posterior laminectomy procedure.

For the lesions above C2 when located ventrally, an extensive lateral approach to the foramen magnum can be used which needs vertebral artery transposition and sigmoid sinus skeletonization; on the other hand, ventral subaxial lesions can be addressed via standard anterior cervical procedure.

Thoracic IDEM tumors are generally operated via posterior approaches due to complications and difficulties of the transthoracic technique described by Bohlman which needs significant lung retraction and may cause serious vascular injuries [16]. Various posterior techniques have been described for ventral thoracic lesion removal including the traditional extracavitary technique described by Larson which is suitable for both ventrally located tumors and tumors with large extraforaminal component and costotransversectomy technique which is also suitable for lateral and ventrolateral lesions but not for tumors located ventrally due to limited surgical view of the contralateral side passing the midline [17].

Most of lumbar and sacral IDEM tumors are operated via posterior approach or its modifications; anterior trans- or retroperitoneal approaches are barely used now.

**49**

*Intradural Extramedullary Spinal Tumors DOI: http://dx.doi.org/10.5772/intechopen.85360*

makes spinal column unstable [19].

distinguishing the motor nerve of origin.

avoid CSF leakage from the incision site.

performed.

The key to a successful surgery is minimal cord or nerve root retraction, and for this purpose extensive resection of bony structures may be necessary, and this might lead to spinal instability. There are multiple reports of successful spinal instru-

Bilateral laminectomy and medial facetectomies usually do not cause spinal instability, while total unilateral facetectomy especially at cervical or lumbar area

Patients undergoing standard posterior approach are placed in prone position with head fixed in Mayfield head holder for cervical tumors or placed at the headrest frame. Arms are placed along side of the trunk in upper thoracic lesions or are

Motor and sensory evoked potential monitoring system is applied, and for cervical and lumbosacral lesions, continuous nerve root EMG monitoring should also be

Midline skin and fascia incisions are made, and classic subperiosteal dissection of the paravertebral muscles is performed. Small laterally located lesions can be reached by a simple hemilaminectomy, but bilateral laminectomy widens the surgical view and may be preferred in most surgeries. Laminectomy length should exceed the whole length of the tumor, and regarding the tumor size and location, unilateral facetectomy may also be performed; intraoperative ultrasonography is

Dura is usually opened posteriorly in a linear fashion which makes duraplasty much easier but also can be opened in a T-shaped fashion or at the paramedian site. Dural opening length should exceed the tumors' length, and when opened, it is sutured to the paraspinal musculature, and then the arachnoid layer is opened. Surgical microscope is mandatory in intradural tumor resection surgeries, and under microscopic view careful dissection of the arachnoid layer, cord, and nerve roots from the tumor is carried out. NSTs are usually originated from dorsal nerve rootlets, but the normal anatomy might be distorted; careful identification of the afferent and efferent origin nerve roots should be performed before tumor resection. Large tumors obstructing the surgical view should be entered and debulked by an ultrasonic aspirator, and then careful identification of the origin roots is carried out. Sensory origin nerve roots are usually bulge and vascular, but motor ones can appear totally normal, and motor evoked potential monitoring can be helpful in

NSTs might extend into the pial surface of the spinal cord, and so, no obvious sensory afferent root might be distinguishable; in these cases, careful dissection of the tumor from the cord substance should be performed. At the cervical and lumbar spine, preservation of the functional motor roots is important, and only those confirmed to be nonfunctional by motor evoked potential studies can be sacrificed. After identification and ligation of the origin afferent and efferent roots with preservation of all functional ones, the tumor is carefully dissected and resected. Subarachnoid space is irrigated vigorously until the blood is cleared. Dural closure is performed with running sutures in a watertight fashion, and then multilayer suturing of the paraspinal muscles, fascia, subcutaneous layer, and skin is carried out. Some surgeons advocate the use of lumbar drain post-op for 48–72 hours to

NSTs can grow extradurally and into neural foramina and even beyond that and get to a significant size at the paraspinal regions; in these cases, we prefer to operate the

helpful in determining the adequacy of the laminectomy extension.

mented fusion surgeries in treated patients with IDEM [18].

**7. Surgical treatment of nerve sheath tumors**

abducted by 90° in lower thoracic or lumbosacral lesions.

#### *Intradural Extramedullary Spinal Tumors DOI: http://dx.doi.org/10.5772/intechopen.85360*

*Brain and Spinal Tumors - Primary and Secondary*

**6. Indications for surgery and surgical routes**

are the major factors determining the surgical route.

addressed via standard anterior cervical procedure.

surgical view of the contralateral side passing the midline [17].

ies in a short period of time.

**Figure 5.**

*ependymoma.*

posterior laminectomy procedure.

Surgical intervention is required in almost all symptomatic patients, but in syndromic patients with mild symptoms, due to higher chance of regrowth and multiple lesions, surgery might not be performed in order to avoid multiple surger-

*Sagittal T2W (A), T1 non-contrast, (B) and contrast-enhanced (C) images of a patient with filum terminale* 

All patients with progressive neural or functional impairment and those with

Asymptomatic patients can be followed by serial clinical and radiological examinations, and surgery is not advised for diagnostic purposes only; the only exception would be myxopapillary ependymoma in which asymptomatic patients may be advised to undergo surgical evacuation for CSF seeding prevention [14, 15].

Most of IDEM tumors can be approached via simple posterior standard laminectomy, though the tumor location in the spinal axis and its relation to the spinal cord

Cervical lesions can be addressed via both anterior and posterior approaches. Tumors located posteriorly, laterally, and ventrolaterally can be approached by

For the lesions above C2 when located ventrally, an extensive lateral approach to the foramen magnum can be used which needs vertebral artery transposition and sigmoid sinus skeletonization; on the other hand, ventral subaxial lesions can be

Thoracic IDEM tumors are generally operated via posterior approaches due to complications and difficulties of the transthoracic technique described by Bohlman which needs significant lung retraction and may cause serious vascular injuries [16]. Various posterior techniques have been described for ventral thoracic lesion removal including the traditional extracavitary technique described by Larson which is suitable for both ventrally located tumors and tumors with large extraforaminal component and costotransversectomy technique which is also suitable for lateral and ventrolateral lesions but not for tumors located ventrally due to limited

Most of lumbar and sacral IDEM tumors are operated via posterior approach or its modifications; anterior trans- or retroperitoneal approaches are barely used now.

rapid tumor growth in serial MR studies should undergo surgery.

**48**

The key to a successful surgery is minimal cord or nerve root retraction, and for this purpose extensive resection of bony structures may be necessary, and this might lead to spinal instability. There are multiple reports of successful spinal instrumented fusion surgeries in treated patients with IDEM [18].

Bilateral laminectomy and medial facetectomies usually do not cause spinal instability, while total unilateral facetectomy especially at cervical or lumbar area makes spinal column unstable [19].

## **7. Surgical treatment of nerve sheath tumors**

Patients undergoing standard posterior approach are placed in prone position with head fixed in Mayfield head holder for cervical tumors or placed at the headrest frame. Arms are placed along side of the trunk in upper thoracic lesions or are abducted by 90° in lower thoracic or lumbosacral lesions.

Motor and sensory evoked potential monitoring system is applied, and for cervical and lumbosacral lesions, continuous nerve root EMG monitoring should also be performed.

Midline skin and fascia incisions are made, and classic subperiosteal dissection of the paravertebral muscles is performed. Small laterally located lesions can be reached by a simple hemilaminectomy, but bilateral laminectomy widens the surgical view and may be preferred in most surgeries. Laminectomy length should exceed the whole length of the tumor, and regarding the tumor size and location, unilateral facetectomy may also be performed; intraoperative ultrasonography is helpful in determining the adequacy of the laminectomy extension.

Dura is usually opened posteriorly in a linear fashion which makes duraplasty much easier but also can be opened in a T-shaped fashion or at the paramedian site. Dural opening length should exceed the tumors' length, and when opened, it is sutured to the paraspinal musculature, and then the arachnoid layer is opened. Surgical microscope is mandatory in intradural tumor resection surgeries, and under microscopic view careful dissection of the arachnoid layer, cord, and nerve roots from the tumor is carried out. NSTs are usually originated from dorsal nerve rootlets, but the normal anatomy might be distorted; careful identification of the afferent and efferent origin nerve roots should be performed before tumor resection. Large tumors obstructing the surgical view should be entered and debulked by an ultrasonic aspirator, and then careful identification of the origin roots is carried out. Sensory origin nerve roots are usually bulge and vascular, but motor ones can appear totally normal, and motor evoked potential monitoring can be helpful in distinguishing the motor nerve of origin.

NSTs might extend into the pial surface of the spinal cord, and so, no obvious sensory afferent root might be distinguishable; in these cases, careful dissection of the tumor from the cord substance should be performed. At the cervical and lumbar spine, preservation of the functional motor roots is important, and only those confirmed to be nonfunctional by motor evoked potential studies can be sacrificed. After identification and ligation of the origin afferent and efferent roots with preservation of all functional ones, the tumor is carefully dissected and resected. Subarachnoid space is irrigated vigorously until the blood is cleared. Dural closure is performed with running sutures in a watertight fashion, and then multilayer suturing of the paraspinal muscles, fascia, subcutaneous layer, and skin is carried out. Some surgeons advocate the use of lumbar drain post-op for 48–72 hours to avoid CSF leakage from the incision site.

NSTs can grow extradurally and into neural foramina and even beyond that and get to a significant size at the paraspinal regions; in these cases, we prefer to operate the

intradural part first and decompress the spinal canal; the extradural part can be evacuated at the same procedure or may be addressed to in a staged surgery via the same route or in another surgical route depending on the size of the extra-foraminal part.

#### **8. Surgical treatment of spinal meningiomas**

In order to approach a spinal meningioma, a surgeon must consider the site and the location of the tumor regarding the cord and boney structures. Meningiomas barely have extradural components and are commonly ventral to the cord.

Cervical meningiomas are addressed by a posterior standard technique if located dorsally or by an anterior approach if located ventrally. Thoracic meningiomas are usually reached via posterior routes, and if located ventrally, extracavitary or costotransversectomy techniques might be used; sectioning and suture rotating the dentate ligament may be helpful for a better and wider surgical view. Lumbar meningiomas are usually operated via posterior approaches because the surgeon is able to safely retract the nerve roots.

Patients are positioned in the same way as patients with NSTs, and motor and sensory evoked potentials are monitored before and during the surgery.

Meningiomas are dural-based lesions, and the extent of dural involvement may be greater than the amount shown in MR studies so the laminectomy length should exceed the cephalad and caudal poles of the tumor, and intraoperative sonography is very helpful in this regard. Durotomy should be performed in an ellipsoid fashion in dorsally located tumors so that the tumor and the involved dura matter could be resected totally.

Ventrally located meningiomas are more challenging both in surgical resection and dural reconstitution which the latter might even be impossible; hence, many prefer dural coagulation instead of resection. Dural coagulation at the tumor base reduces intraoperative bleeding.

When the tumor poles are exposed, the dentate ligament can be sectioned and rotated by a suture for surgical view improvement. Large tumors compressing the cord should be debulked by an ultrasonic aspirator before resection so that spinal cord gets decompressed and a better view of the tumor margins could be achieved.

#### **9. Surgical treatment of filum terminale ependymomas**

Myxopapillary ependymomas are solid fleshy lesions originating from filum terminale and can have a large size at the time of diagnosis. These lesions are exclusively approached via posterior surgical procedure, and care must be taken to resect these tumors in an en bloc fashion so that CSF seeding and metastasis would not occur [20, 21].

Standard posterior approach in a prone position is performed under electrophysiologic monitoring evaluation, and when laminectomy is completed, dura is opened dorsally in a linear fashion and then sutured to paraspinal musculature. Arachnoid layer is opened, and careful microdissection of the neural roots from tumor is performed, and filum is identified proximal and distal to the tumor and tested by a neurostimulator.

Filum is cauterized and sectioned at both ends of the tumor, and then en bloc tumor resection is achieved with caution not to retract adjacent nerve roots excessively.

In some cases, en bloc resection of the tumor cannot be obtained especially when the tumor is too fragile and falls apart even with most careful microdissection or

**51**

**Author details**

Saleh Rasras\* and Arash Kiani

provided the original work is properly cited.

*Intradural Extramedullary Spinal Tumors DOI: http://dx.doi.org/10.5772/intechopen.85360*

**10. Adjuvant therapies**

tumor growth [23, 24].

and subtotal resection would be the only option.

to face less surgical procedures in their lifetime.

closure of the overlying compartments is carried out.

when it's too large, and safe en bloc resection is impossible. The presence of functional nerve roots in the tumor substance also makes en bloc resection impossible,

Dura is approximated by running sutures in a watertight fashion, and multilayer

Adjuvant therapies do not play a major role in treatment of IDEM tumors, and microsurgical gross total resection still is the gold standard modality of treatment. Radiotherapy has a defined role in patients with myxopapillary ependymoma and improves their progression-free survival when administered postsurgery [21]. Radiotherapy is administered in multiple recurrent meningiomas or the ones with atypical or malignant histology [22]. Stereotactic radiosurgery has been shown to be beneficial in patients with NSTs or meningiomas as the primary modality of treatment or as an adjuvant therapy in patients with post-operation radiologic

Stereotactic radiosurgery is of more importance in syndromic patients who might have multiple lesions and also a higher tumor progression rate and helps them

© 2019 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium,

Ahvaz Jundishapour University of Medical Sciences, Ahvaz, Iran

\*Address all correspondence to: dr.rasras@gmail.com

#### *Intradural Extramedullary Spinal Tumors DOI: http://dx.doi.org/10.5772/intechopen.85360*

when it's too large, and safe en bloc resection is impossible. The presence of functional nerve roots in the tumor substance also makes en bloc resection impossible, and subtotal resection would be the only option.

Dura is approximated by running sutures in a watertight fashion, and multilayer closure of the overlying compartments is carried out.

## **10. Adjuvant therapies**

*Brain and Spinal Tumors - Primary and Secondary*

**8. Surgical treatment of spinal meningiomas**

able to safely retract the nerve roots.

reduces intraoperative bleeding.

resected totally.

not occur [20, 21].

tested by a neurostimulator.

intradural part first and decompress the spinal canal; the extradural part can be evacuated at the same procedure or may be addressed to in a staged surgery via the same route or in another surgical route depending on the size of the extra-foraminal part.

In order to approach a spinal meningioma, a surgeon must consider the site and the location of the tumor regarding the cord and boney structures. Meningiomas barely have extradural components and are commonly ventral to the cord.

Cervical meningiomas are addressed by a posterior standard technique if located

Patients are positioned in the same way as patients with NSTs, and motor and

Meningiomas are dural-based lesions, and the extent of dural involvement may be greater than the amount shown in MR studies so the laminectomy length should exceed the cephalad and caudal poles of the tumor, and intraoperative sonography is very helpful in this regard. Durotomy should be performed in an ellipsoid fashion in dorsally located tumors so that the tumor and the involved dura matter could be

Ventrally located meningiomas are more challenging both in surgical resection and dural reconstitution which the latter might even be impossible; hence, many prefer dural coagulation instead of resection. Dural coagulation at the tumor base

When the tumor poles are exposed, the dentate ligament can be sectioned and rotated by a suture for surgical view improvement. Large tumors compressing the cord should be debulked by an ultrasonic aspirator before resection so that spinal cord gets decompressed and a better view of the tumor margins could be achieved.

Myxopapillary ependymomas are solid fleshy lesions originating from filum terminale and can have a large size at the time of diagnosis. These lesions are exclusively approached via posterior surgical procedure, and care must be taken to resect these tumors in an en bloc fashion so that CSF seeding and metastasis would

Standard posterior approach in a prone position is performed under electrophysiologic monitoring evaluation, and when laminectomy is completed, dura is opened dorsally in a linear fashion and then sutured to paraspinal musculature. Arachnoid layer is opened, and careful microdissection of the neural roots from tumor is performed, and filum is identified proximal and distal to the tumor and

Filum is cauterized and sectioned at both ends of the tumor, and then en bloc tumor resection is achieved with caution not to retract adjacent nerve roots

In some cases, en bloc resection of the tumor cannot be obtained especially when the tumor is too fragile and falls apart even with most careful microdissection or

sensory evoked potentials are monitored before and during the surgery.

**9. Surgical treatment of filum terminale ependymomas**

dorsally or by an anterior approach if located ventrally. Thoracic meningiomas are usually reached via posterior routes, and if located ventrally, extracavitary or costotransversectomy techniques might be used; sectioning and suture rotating the dentate ligament may be helpful for a better and wider surgical view. Lumbar meningiomas are usually operated via posterior approaches because the surgeon is

**50**

excessively.

Adjuvant therapies do not play a major role in treatment of IDEM tumors, and microsurgical gross total resection still is the gold standard modality of treatment.

Radiotherapy has a defined role in patients with myxopapillary ependymoma and improves their progression-free survival when administered postsurgery [21].

Radiotherapy is administered in multiple recurrent meningiomas or the ones with atypical or malignant histology [22]. Stereotactic radiosurgery has been shown to be beneficial in patients with NSTs or meningiomas as the primary modality of treatment or as an adjuvant therapy in patients with post-operation radiologic tumor growth [23, 24].

Stereotactic radiosurgery is of more importance in syndromic patients who might have multiple lesions and also a higher tumor progression rate and helps them to face less surgical procedures in their lifetime.

## **Author details**

Saleh Rasras\* and Arash Kiani Ahvaz Jundishapour University of Medical Sciences, Ahvaz, Iran

\*Address all correspondence to: dr.rasras@gmail.com

© 2019 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

## **References**

[1] Jaffe HL. Tumors and Tumorous Conditions of Bones and Joints. Philadelphia: Lea & Febiger; 1958

[2] Akram H, Allibone J. Spinal surgery for palliation in malignant spinal cord compression. Clinical Oncology (Royal College of Radiologists). 2010;**22**:792-800

[3] Maccauro G, Spinelli MS, Mauro S, et al. Physiopathology of spine metastasis. International Journal of Surgical Oncology. 2011;**2011**:107969

[4] Fox MW, Onofrio BM. The natural history and management of symptomatic and asymptomatic vertebral hemangiomas. Journal of Neurosurgery. 1993;**78**:36-45

[5] Traul DE, Shaffrey ME, Schiff D. Part I: Spinal-cord neoplasms– intradural neoplasms. The Lancet Oncology. 2007;**8**:35-45

[6] Gottfried ON, Gluf W, Quinones-Hinojosa A, et al. Spinal meningiomas: Surgical management and outcome. Neurosurgical Focus. 2003;**14**(6):e2

[7] Schaller B. Spinal meningioma: Relationship between histological subtypes and surgical outcome? Journal of Neuro-Oncology. 2005;**75**:157-161

[8] Preston-Martin S. Descriptive epidemiology of primary tumors of the spinal cord and spinal meninges in Los Angeles County, 1972-1985. Neuroepidemiology. 1990;**9**:106-111

[9] Seppälä MT, Haltia MJ, Sankila RJ, et al. Long-term outcome after removal of spinal schwannoma: A clinicopathological study of 187 cases. Journal of Neurosurgery. 1995;**83**:621-626

[10] Kim P, Ebersold MJ, Onofrio BM, et al. Surgery of spinal nerve schwannoma. Risk of neurological deficit after resection of involved root. Journal of Neurosurgery. 1989;**71**:810-814

[11] Requena L, Sangüeza OP. Benign neoplasms with neural differentiation: A review. The American Journal of Dermatopathology. 1995;**17**:75-96

[12] Sonneland PR, Scheithauer BW, Onofrio BM. Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer. 1985;**56**:883-893

[13] National Institutes of Health Consensus Development Conference Statement: Neurofibromatosis. Bethesda, Md., USA, July 13-15, 1987. Neurofibromatosis. 1988;**1**:172-178

[14] Mridha AR, Sharma MC, Sarkar C, et al. Myxopapillary ependymoma of lumbosacral region with metastasis to both cerebellopontine angles: Report of a rare case. Child's Nervous System. 2007;**24**:1209-1213

[15] Fassett DR, Pingree J, Kestle JRW, et al. The high incidence of tumor dissemination in myxopapillary ependymoma in pediatric patients. Report of five cases and review of the literature. Journal of Neurosurgery. 2005;**102**(1 suppl):59-64

[16] Bohlman HH, Zdeblick TA. Anterior excision of herniated thoracic discs. The Journal of Bone and Joint Surgery. American Volume. 1988;**20**:1038-1047

[17] McCormick PC. Surgical management of dumbbell and paraspinal tumors of the thoracic and lumbar spine. Neurosurgery. 1996;**38**:67-74

[18] Ando K, Imagama S, Ito Z, et al. Unilateral instrumented fixation for cervical dumbbell tumors. Journal of

**53**

*Intradural Extramedullary Spinal Tumors DOI: http://dx.doi.org/10.5772/intechopen.85360*

Orthopaedic Surgery and Research.

[19] Abumi K, Panjabi M, Kramer K, et al. Biomechanical evaluation of lumbar spinal stability after graded facetectomies. Spine. 1990;**15**:1142-1147

[20] Wen BC, Hussey DH, Hitchon PW, et al. The role of radiation therapy in the management of ependymomas of the spinal cord. International Journal of Radiation Oncology, Biology, Physics.

[21] Akyurek S, Chang EL, Yu TK, et al. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. Neuro-

Kamnerdsupaphon P, et al. CyberKnife radiosurgery for benign intradural extramedullary spinal tumors. Neurosurgery. 2006;**58**:674-685

[24] Gerszten PC, Burton SA, Ozhasoglu C, et al. Radiosurgery for benign intradural spinal tumors. Neurosurgery.

Oncology. 2006;**80**:177-183

Oncologica. 1997;**36**:88-90

[23] Dodd RL, Ryu MR,

2008;**62**:887-896

[22] Schiebe ME, Hoffmann W, Kortmann RD, et al. Radiotherapy in recurrent malignant meningiomas with multiple spinal manifestations. Acta

2014;**9**:2

1991;**20**:781-786

*Intradural Extramedullary Spinal Tumors DOI: http://dx.doi.org/10.5772/intechopen.85360*

Orthopaedic Surgery and Research. 2014;**9**:2

[19] Abumi K, Panjabi M, Kramer K, et al. Biomechanical evaluation of lumbar spinal stability after graded facetectomies. Spine. 1990;**15**:1142-1147

[20] Wen BC, Hussey DH, Hitchon PW, et al. The role of radiation therapy in the management of ependymomas of the spinal cord. International Journal of Radiation Oncology, Biology, Physics. 1991;**20**:781-786

[21] Akyurek S, Chang EL, Yu TK, et al. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. Neuro-Oncology. 2006;**80**:177-183

[22] Schiebe ME, Hoffmann W, Kortmann RD, et al. Radiotherapy in recurrent malignant meningiomas with multiple spinal manifestations. Acta Oncologica. 1997;**36**:88-90

[23] Dodd RL, Ryu MR, Kamnerdsupaphon P, et al. CyberKnife radiosurgery for benign intradural extramedullary spinal tumors. Neurosurgery. 2006;**58**:674-685

[24] Gerszten PC, Burton SA, Ozhasoglu C, et al. Radiosurgery for benign intradural spinal tumors. Neurosurgery. 2008;**62**:887-896

**52**

1995;**83**:621-626

*Brain and Spinal Tumors - Primary and Secondary*

schwannoma. Risk of neurological deficit after resection of involved root. Journal of Neurosurgery.

[11] Requena L, Sangüeza OP. Benign neoplasms with neural differentiation: A review. The American Journal of Dermatopathology. 1995;**17**:75-96

[12] Sonneland PR, Scheithauer BW, Onofrio BM. Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases.

[13] National Institutes of Health Consensus Development Conference Statement: Neurofibromatosis. Bethesda, Md., USA, July 13-15, 1987. Neurofibromatosis. 1988;**1**:172-178

[14] Mridha AR, Sharma MC, Sarkar C, et al. Myxopapillary ependymoma of lumbosacral region with metastasis to both cerebellopontine angles: Report of a rare case. Child's Nervous System.

[15] Fassett DR, Pingree J, Kestle JRW, et al. The high incidence of tumor dissemination in myxopapillary ependymoma in pediatric patients. Report of five cases and review of the literature. Journal of Neurosurgery.

[16] Bohlman HH, Zdeblick TA. Anterior excision of herniated thoracic discs. The Journal of Bone and Joint Surgery. American Volume. 1988;**20**:1038-1047

Cancer. 1985;**56**:883-893

2007;**24**:1209-1213

2005;**102**(1 suppl):59-64

[17] McCormick PC. Surgical management of dumbbell and paraspinal tumors of the thoracic and lumbar spine. Neurosurgery.

[18] Ando K, Imagama S, Ito Z, et al. Unilateral instrumented fixation for cervical dumbbell tumors. Journal of

1996;**38**:67-74

1989;**71**:810-814

[1] Jaffe HL. Tumors and Tumorous Conditions of Bones and Joints. Philadelphia: Lea & Febiger; 1958

[2] Akram H, Allibone J. Spinal surgery for palliation in malignant spinal cord compression. Clinical Oncology (Royal College of Radiologists).

[3] Maccauro G, Spinelli MS, Mauro S, et al. Physiopathology of spine metastasis. International Journal of Surgical Oncology. 2011;**2011**:107969

[4] Fox MW, Onofrio BM. The natural history and management of symptomatic and asymptomatic vertebral hemangiomas. Journal of Neurosurgery. 1993;**78**:36-45

[5] Traul DE, Shaffrey ME, Schiff D. Part I: Spinal-cord neoplasms– intradural neoplasms. The Lancet

[6] Gottfried ON, Gluf W, Quinones-Hinojosa A, et al. Spinal meningiomas: Surgical management and outcome. Neurosurgical Focus. 2003;**14**(6):e2

[7] Schaller B. Spinal meningioma: Relationship between histological subtypes and surgical outcome? Journal of Neuro-Oncology. 2005;**75**:157-161

[8] Preston-Martin S. Descriptive epidemiology of primary tumors of the spinal cord and spinal meninges in Los Angeles County, 1972-1985. Neuroepidemiology. 1990;**9**:106-111

[9] Seppälä MT, Haltia MJ, Sankila RJ, et al. Long-term outcome after removal of spinal schwannoma: A clinicopathological study of 187 cases. Journal of Neurosurgery.

[10] Kim P, Ebersold MJ, Onofrio BM, et al. Surgery of spinal nerve

Oncology. 2007;**8**:35-45

2010;**22**:792-800

**References**

**55**

**Chapter 4**

**Abstract**

Jugular Foramen Paragangliomas

*Bernardo Alves Barbosa, Diogo Fabricio Coelho de Melo,* 

Jugular foramen paragangliomas are rare neoplasms occurring with a myriad of symptoms originating from paraganglionic tissue derived from the neural crest, comprising about 0.03% of all human tumors. Patients usually present with symptoms of dysfunction of VI, VII, VIII, IX, X, XI, XII nerves and sympathetic trunk. Depending on the tumor's topography, various approaches might be used to obtain its gross total resection. Jugular Foramen's paraganglioma classification, nuances of the approaches, pathology, postoperative complications, and outcomes are revised as follows. In conclusion, anatomical knowledge and the disease's comprehension are essential when dealing with such tumors, and despite their rarity, we must be obstinately committed to the surgical technique and devoted to the patient's func-

Tumors located in the jugular foramen are rare, being one of the significant challenges in the surgical practice for cranial base neurosurgeons. Several tumors can affect this region, among them schwannomas, paragangliomas, and meningiomas representing the most common. Head and neck paragangliomas are rare neoplasms comprising about 0.03% of all human tumors. The yearly incidence is estimated to be at around 0.001% [1, 2]. Rarely, tumors located in the jugular foramen show intracranial and extracranial extension and, thus, present a myriad of symptoms, with several clinical syndromes described in the literature (see clinical presentation). The term "glomus tumor" has been used to describe the most common tumor related to this region, representing a tumor originating from paraganglionic tissue derived from the neural crest whose cells have the capacity to reserve and release catecholamines and may have clinical implications (see preoperative

Malignant tumors can also affect the jugular foramen, including metastatic tumors (carcinomas), chondrosarcomas and chordomas, as part of the differential diagnosis of these lesions [3]. The detailed discussion of the differential diagnosis of these lesions

is not part of the scope of this chapter and can be seen in other references [3-5].

*Carolina Salviano de Abreu Nery, Fred Bernardes Filho*

*Breno Nery, Rodrigo Antônio Fernandes Costa,* 

*Eduardo Quaggio, Ricardo Lopes Araújo,* 

*and George Peter Stevens*

tional postoperative outcome.

**1. Introduction**

preparation) [3, 4].

**Keywords:** paraganglioma, brain neoplasms

## **Chapter 4**

## Jugular Foramen Paragangliomas

*Breno Nery, Rodrigo Antônio Fernandes Costa, Eduardo Quaggio, Ricardo Lopes Araújo, Bernardo Alves Barbosa, Diogo Fabricio Coelho de Melo, Carolina Salviano de Abreu Nery, Fred Bernardes Filho and George Peter Stevens*

## **Abstract**

Jugular foramen paragangliomas are rare neoplasms occurring with a myriad of symptoms originating from paraganglionic tissue derived from the neural crest, comprising about 0.03% of all human tumors. Patients usually present with symptoms of dysfunction of VI, VII, VIII, IX, X, XI, XII nerves and sympathetic trunk. Depending on the tumor's topography, various approaches might be used to obtain its gross total resection. Jugular Foramen's paraganglioma classification, nuances of the approaches, pathology, postoperative complications, and outcomes are revised as follows. In conclusion, anatomical knowledge and the disease's comprehension are essential when dealing with such tumors, and despite their rarity, we must be obstinately committed to the surgical technique and devoted to the patient's functional postoperative outcome.

**Keywords:** paraganglioma, brain neoplasms

#### **1. Introduction**

Tumors located in the jugular foramen are rare, being one of the significant challenges in the surgical practice for cranial base neurosurgeons. Several tumors can affect this region, among them schwannomas, paragangliomas, and meningiomas representing the most common. Head and neck paragangliomas are rare neoplasms comprising about 0.03% of all human tumors. The yearly incidence is estimated to be at around 0.001% [1, 2]. Rarely, tumors located in the jugular foramen show intracranial and extracranial extension and, thus, present a myriad of symptoms, with several clinical syndromes described in the literature (see clinical presentation). The term "glomus tumor" has been used to describe the most common tumor related to this region, representing a tumor originating from paraganglionic tissue derived from the neural crest whose cells have the capacity to reserve and release catecholamines and may have clinical implications (see preoperative preparation) [3, 4].

Malignant tumors can also affect the jugular foramen, including metastatic tumors (carcinomas), chondrosarcomas and chordomas, as part of the differential diagnosis of these lesions [3]. The detailed discussion of the differential diagnosis of these lesions is not part of the scope of this chapter and can be seen in other references [3-5].

Advances in diagnostic imaging and surgical technique have allowed the understanding of these tumors and their exeresis with lower morbidity and mortality. A brief review of the clinical, diagnostic, imaging, histopathological and surgical aspects related to the glomus tumors of the jugular foramen is given below.

## **2. Clinical presentation**

Glomus tumors of the jugular foramen present with slow growth and with early signs and clinical symptoms, being diagnosed on average after 5 years of onset of symptoms. These tumors have an average growth rate of approximately 1 mm per year [6]. The symptoms are directly related to the site of involvement and infiltration. Tumors of glomus jugulare represent neoplastic lesions that originate in the adventitia of the jugular vein and most commonly present with symptoms related to the involvement of lower cranial nerves, such as vagus (X), accessory (XI) and hypoglossus (XII). In the variant of glomus tympanicum, which are tumors related to the Jacobson's nerve, the most common initial clinical presentation is the presence of tinnitus, followed by conductive deafness and vertigo. Jacobson's nerve represents a tympanic branch of the glossopharyngeal nerve, which conveys the sensitivity of the tympanic membrane, auditory tube, and mastoid region. In the third anatomotopographic variety of this tumor we have the glomus vagale, originating from Arnold's nerve. Arnold's nerve emerges between the superior and inferior ganglia of the vagus nerve (auricular branch of the vagus nerve) and

#### **Figure 1.**

*On the left, anatomical relationships of the glomus tumor of the jugular foramen; on the right, otoscopy revealing the presence of a tumor in the lower right field.*


**57**

**Figure 2.**

*Jugular Foramen Paragangliomas*

*DOI: http://dx.doi.org/10.5772/intechopen.84232*

locations are described in **Table 1**.

**3. Pre-operative diagnosis and image classification**

is responsible for the sensitive innervation of the skin over the outer ear's shell. Detailed examination through otoscopy may reveal the presence of tympanic membrane invasion, and otorhinolaryngology may be evidenced in some cases (**Figure 1**). Classical syndromes related to this type of tumor and their respective

Detailed clinical examination is essential for accurate lesion location and scheduling of resection of the intra- and extracranial portions of the tumor. Detailed examinations of the functions of VI, VII, VIII, IX, X, XI, XII and sympathetic trunk should be performed, seeking to predict the intraoperative relations of the tumor with the cranial nerves. Prior to the decision to resect the lesion, evaluation of lesion growth pattern through serial imaging is not considered bad practice. Computed tomography (CT) scans and fine sections (1.0 mm) with reconstruction in the coronal and sagittal planes are essential to delineate the bone relations of the tumor during the chosen surgical approach, as well as the study by the magnetic resonance imaging (MRI) with gadolinium is essential for the evaluation of the neurovascular relationships of the lesion. Tomography can show a smoother surface and have associated bone erosion in cases of schwannoma of the jugular foramen, in contrast to paragangliomas of this region, which demonstrate a more irregular

*On the superior left, glomus tumor of the right jugular foramen seen on the tomography (moth-eaten pattern); on the superior right, magnetic nuclear resonance with "salt and pepper" appearance; below, angiography evidencing irrigation of a glomus tumor of the head predominantly by the right ascending pharyngeal artery.*

#### **Table 1.**

*Syndromes related to the jugular foramen.*

#### *Jugular Foramen Paragangliomas DOI: http://dx.doi.org/10.5772/intechopen.84232*

*Brain and Spinal Tumors - Primary and Secondary*

**2. Clinical presentation**

Advances in diagnostic imaging and surgical technique have allowed the understanding of these tumors and their exeresis with lower morbidity and mortality. A brief review of the clinical, diagnostic, imaging, histopathological and surgical aspects related to the glomus tumors of the jugular foramen is given below.

Glomus tumors of the jugular foramen present with slow growth and with early signs and clinical symptoms, being diagnosed on average after 5 years of onset of symptoms. These tumors have an average growth rate of approximately 1 mm per year [6]. The symptoms are directly related to the site of involvement and infiltration. Tumors of glomus jugulare represent neoplastic lesions that originate in the adventitia of the jugular vein and most commonly present with symptoms related to the involvement of lower cranial nerves, such as vagus (X), accessory (XI) and hypoglossus (XII). In the variant of glomus tympanicum, which are tumors related to the Jacobson's nerve, the most common initial clinical presentation is the presence of tinnitus, followed by conductive deafness and vertigo. Jacobson's nerve represents a tympanic branch of the glossopharyngeal nerve, which conveys the sensitivity of the tympanic membrane, auditory tube, and mastoid region. In the third anatomotopographic variety of this tumor we have the glomus vagale, originating from Arnold's nerve. Arnold's nerve emerges between the superior and inferior ganglia of the vagus nerve (auricular branch of the vagus nerve) and

**56**

**Table 1.**

**Figure 1.**

**Jugular foramen syndromes and respective affected nerves**

*revealing the presence of a tumor in the lower right field.*

*Syndromes related to the jugular foramen.*

**Vernet Collet-**

*On the left, anatomical relationships of the glomus tumor of the jugular foramen; on the right, otoscopy* 

IX + + +

**Sicard**

X + + + + + + XI + + + +/− + + XII — + + + + — Sympathetic fibers — — + +/− — +

**Vilaret Tapia Jackson Schmidt**

is responsible for the sensitive innervation of the skin over the outer ear's shell. Detailed examination through otoscopy may reveal the presence of tympanic membrane invasion, and otorhinolaryngology may be evidenced in some cases (**Figure 1**). Classical syndromes related to this type of tumor and their respective locations are described in **Table 1**.

## **3. Pre-operative diagnosis and image classification**

Detailed clinical examination is essential for accurate lesion location and scheduling of resection of the intra- and extracranial portions of the tumor. Detailed examinations of the functions of VI, VII, VIII, IX, X, XI, XII and sympathetic trunk should be performed, seeking to predict the intraoperative relations of the tumor with the cranial nerves. Prior to the decision to resect the lesion, evaluation of lesion growth pattern through serial imaging is not considered bad practice. Computed tomography (CT) scans and fine sections (1.0 mm) with reconstruction in the coronal and sagittal planes are essential to delineate the bone relations of the tumor during the chosen surgical approach, as well as the study by the magnetic resonance imaging (MRI) with gadolinium is essential for the evaluation of the neurovascular relationships of the lesion. Tomography can show a smoother surface and have associated bone erosion in cases of schwannoma of the jugular foramen, in contrast to paragangliomas of this region, which demonstrate a more irregular

#### **Figure 2.**

*On the superior left, glomus tumor of the right jugular foramen seen on the tomography (moth-eaten pattern); on the superior right, magnetic nuclear resonance with "salt and pepper" appearance; below, angiography evidencing irrigation of a glomus tumor of the head predominantly by the right ascending pharyngeal artery.*

tumoral surface with adjacent bone destruction (**Figure 1**). Neurovascular relationships with the internal carotid artery, cephalic trunk, as well as its intra and extracranial extension are better visualized through the MRI. In T1 weighted images, the glomus tumor is hypo/isointense to the brainstem, and gadolinium injection presents the classic salt and pepper enhancement pattern (**Figure 1**).

Pepper's image represents the hypointense void sign, and the "salt" image represents the hyperintense signal caused by low vascular flow or intratumoral subacute hemorrhage. These tumors typically have a distinct pattern of infiltration, generally following pathways of lower resistance, such as air mastoid cells, vascular channels, Eustachian tube and neural foramina [7, 8].

Angioresonance, angiotomography, or venography may help to demonstrate the type of vascularization of the tumor and its local venous circulation (**Figure 2**). Digital angiography is a prerequisite in patients with extremely vascular lesions, for whom preoperative embolization is necessary to reduce bleeding during surgery. **Table 2** shows the imaging modalities and the peculiar characteristics of the tumors of this region during the preoperative study, aiming to differentiate the three most common lesions of this region. A balloon occlusion test should be performed in case of involvement of the internal carotid artery.

The most relevant laboratory exams prior to surgery are serum and urinary catecholamines, as well as urinary levels of vanilmandelic acid and urinary metanephrines, to determine the possibility of neuroendocrine secretion of the tumor. Five percent of glomus tumors of the jugular foramen (JF) are secretory, and in


**59**

*Jugular Foramen Paragangliomas*

temporal bone

the petrous apex

**Table 3.**

**Table 4.**

*DOI: http://dx.doi.org/10.5772/intechopen.84232*

A Tumors limited to the space of the middle ear

*Fisch classification for glomus tumors of temporal region.*

present intracranial extension

intra and extracranial involvement [11].

**4. Histopathological characteristics**

cells that perform tumor irrigation (**Figure 3**).

**5. Surgical approaches**

these cases, the preoperative use of alpha and beta-blockers are essential to avoid complications. The most commonly used radiological classification in the preoperative evaluation of patients with glomus tumors of the jugular foramen are Fisch [9] (**Table 3**) and Glasscock-Jackson [10] (**Table 4**). The most used classification of schwannomas of the jugular foramen is Samii's classification, who divided the schwannomas of the jugular foramen into four groups: type A which represents the primary tumors of the cerebellar angle with minimal enlargement of the JF; type B that are the primary tumors of the JF with intracranial extension; type C which have extracranial tumors with extension to the jugular foramen (with clinical signs of involvement of the XII nerve); and type D being the "hourglass" tumors with

IV Tumor extending beyond the petrous apex to the infratemporal clivus or infratemporal fossa; may

II Tumor extending below the internal acoustic meatus; may present intracranial extension

III Tumor extending to the petrous apex; may present intracranial extension

B Tumors limited to the middle ear or mastoid, without involvement of the infralabirintic space of the

C Tumor involving the infralabirintic space and apical spaces of the temporal bone, with extension to

C1 Tumor with little involvement of the vertical portion of the carotid canal

C2 Tumor invading the vertical portion of the carotid canal C3 Tumor invading the horizontal portion of the carotid canal D1 Tumor invading the horizontal portion of the carotid canal D2 Tumor with intracranial extension >2 cm in diameter

I Tumor involving jugular bulb, middle ear and mastoid

*Glasscock-Jackson classification for glomus tumors of jugular foramen.*

Glomus tumors of the jugular foramen present polygonal epithelioid cells with clear and abundant cytoplasm arranged in small lobes (alveolar arrangement). These cellular clusters were given the name Zellballen, which means "cellular balls" in German. Numerous capillaries can also be observed in the proximity of tumor

Patients should be routinely monitored, and preoperative antibiotic (30 min before incision) should be administered. The ideal approach for each patient should be chosen after a meticulous preoperative study of lesion's location. Tumors that

#### **Table 2.**

*Differential diagnoses by imaging of the main lesions affecting the jugular foramen.*

#### *Jugular Foramen Paragangliomas DOI: http://dx.doi.org/10.5772/intechopen.84232*


#### **Table 3.**

*Brain and Spinal Tumors - Primary and Secondary*

Eustachian tube and neural foramina [7, 8].

of involvement of the internal carotid artery.

• "Moth-eaten" pattern of temporal bone • Dehiscence of the floor of the tympanic cavity • Erosion of the ossicular

• Enlargement of the jugular foramen without destruction

and homogeneous contrast enhancement • Hyperostosis, intralesional calcifications

*Differential diagnoses by imaging of the main lesions affecting the jugular foramen.*

chain

Schwannoma • Isodense tumors

Meningioma • Isodense with intense

**Tumor type and radiological features by imaging studies**

Glomus tumors

tumoral surface with adjacent bone destruction (**Figure 1**). Neurovascular relationships with the internal carotid artery, cephalic trunk, as well as its intra and extracranial extension are better visualized through the MRI. In T1 weighted images, the glomus tumor is hypo/isointense to the brainstem, and gadolinium injection

Pepper's image represents the hypointense void sign, and the "salt" image represents the hyperintense signal caused by low vascular flow or intratumoral subacute hemorrhage. These tumors typically have a distinct pattern of infiltration, generally following pathways of lower resistance, such as air mastoid cells, vascular channels,

Angioresonance, angiotomography, or venography may help to demonstrate the type of vascularization of the tumor and its local venous circulation (**Figure 2**). Digital angiography is a prerequisite in patients with extremely vascular lesions, for whom preoperative embolization is necessary to reduce bleeding during surgery. **Table 2** shows the imaging modalities and the peculiar characteristics of the tumors of this region during the preoperative study, aiming to differentiate the three most common lesions of this region. A balloon occlusion test should be performed in case

The most relevant laboratory exams prior to surgery are serum and urinary catecholamines, as well as urinary levels of vanilmandelic acid and urinary metanephrines, to determine the possibility of neuroendocrine secretion of the tumor. Five percent of glomus tumors of the jugular foramen (JF) are secretory, and in

**resonance**

• T1 weighted images heterogeneously enhanced with gadolinium "salt and pepper" pattern

• T1 hypointense, T2 hyperintense and moderate enhancement with

• Characteristic and homogeneous enhancement • Presence of dural tail

gadolinium

**Digital angiography**

• Irrigation of the inferomedial portion of the tumor by the ascending pharyngeal artery • Posterior auricular, stylomastoid and • occipital arteries irrigate the posterolateral portion of the tumor • Internal maxillary artery and ACI may contribute to larger tumors

• Absence of significant irrigation or compression of the jugular vein

• Early enhancement with slow emptying

**Computed tomography Nuclear magnetic** 

presents the classic salt and pepper enhancement pattern (**Figure 1**).

**58**

**Table 2.**

*Fisch classification for glomus tumors of temporal region.*


#### **Table 4.**

*Glasscock-Jackson classification for glomus tumors of jugular foramen.*

these cases, the preoperative use of alpha and beta-blockers are essential to avoid complications. The most commonly used radiological classification in the preoperative evaluation of patients with glomus tumors of the jugular foramen are Fisch [9] (**Table 3**) and Glasscock-Jackson [10] (**Table 4**). The most used classification of schwannomas of the jugular foramen is Samii's classification, who divided the schwannomas of the jugular foramen into four groups: type A which represents the primary tumors of the cerebellar angle with minimal enlargement of the JF; type B that are the primary tumors of the JF with intracranial extension; type C which have extracranial tumors with extension to the jugular foramen (with clinical signs of involvement of the XII nerve); and type D being the "hourglass" tumors with intra and extracranial involvement [11].

## **4. Histopathological characteristics**

Glomus tumors of the jugular foramen present polygonal epithelioid cells with clear and abundant cytoplasm arranged in small lobes (alveolar arrangement). These cellular clusters were given the name Zellballen, which means "cellular balls" in German. Numerous capillaries can also be observed in the proximity of tumor cells that perform tumor irrigation (**Figure 3**).

## **5. Surgical approaches**

Patients should be routinely monitored, and preoperative antibiotic (30 min before incision) should be administered. The ideal approach for each patient should be chosen after a meticulous preoperative study of lesion's location. Tumors that

**Figure 3.**

*Typical alveolar pattern with presence of Zellballen, which are classic epithelioid clusters of these tumors.*

are primarily intracranial located (Samii type A schwannomas) or tumors with a more significant intracranial extension may be approached by the classic lateral suboccipital retrosigmoid approach. The patient should be ideally positioned in a semi-sited position, since the presence of tumor bleeding gravitates downward, maintaining the surgical field with better visibility throughout the resection. Ideally, central venous access and pre-cordial Doppler should be used to prevent and treat air embolism. Pneumatic compression boots should be used to facilitate venous return. The head should be rotated about 30° to the same side of the lesion, aiming a straight direction in relation to the jugular foramen, as well as a smaller cerebellar retraction, and be fixed in a Mayfield head holder. Discrete flexion helps to expose the suboccipital region, facilitating the positioning of the surgeon during the approach. Vigorous rotation and flexion should be avoided as they may compromise jugular venous return; so a space of two fingers should separate the chin from the ipsilateral clavicle for this purpose. A cutaneous incision should be made with an upper limit on the pinna to the posterior musculature of the neck, maintaining a distance of about 3 cm from the mastoid. After incision of the muscular plane, suboccipital craniectomy is performed, ideally exposing the inferior portion of the transverse sinus and medial portion of the sigmoid sinus. The most crucial step of the craniectomy is its inferior extension to the posterior border of the magnum foramen. The dura mater should be cut in a "C" fashion with its convex portion of the cut close to the transverse and sigmoid sinuses. The dura mater, when cut in this way, not only protects the cerebellar hemisphere from contusions but also prevents dural redundancy in the visual field of the surgeon, also allowing more adequate closure at the end of the procedure.

Next, the arachnoid trabeculae of the Magna cistern of the pontocerebellar angle (PCA) should be cut, and careful aspiration of cerebrospinal fluid should follow, allowing relaxation of the neurovascular structures. Then, the anterior and medial portion of the cerebellum must be carefully covered with cottonoids and retracted medially and superiorly with a spatula, and then fixed in a static position. Dialogue with neurophysiologists is essential during this time of surgery, and repositioning of the spatula may be necessary in case of disturbed auditory brainstem evoked potential. It is essential to determine the exact position of the tumor in relation to the sigmoid sinus and bulb of the jugular vein, because in larger tumors of this region the wall of these vessels may be compromised, with catastrophic bleeding that is difficult to control. Extradural drilling of the jugular foramen helps to define the margins of the tumor, and after this maneuver follows the careful debulking of the tumor.

We carefully proceed with the dissection between the tumor and the lower cranial nerves, as well as its separation of the sigmoid sinus and bulb of the jugular vein when they are involved. The use of ultrasonic aspiration helps significantly during

**61**

**Figure 4.**

*petrosal approach.*

*Jugular Foramen Paragangliomas*

*DOI: http://dx.doi.org/10.5772/intechopen.84232*

the resection of these tumors. As the tumor resection is performed, we maintain an incessant dialogue with the neurophysiologist observing the changes during the monitoring of the lower cranial nerves (IX, X, XI, XII). At the end of resection, hemostasis is followed, and defects in the skull base and mastoid should be covered with autologous fat and fibrin glue. The closure in subsequent layers is done, and extubation is monitored by an anesthesiologist in the operating room when there is no cranial nerve injury. Larger tumors (schwannomas B, C, and D of Samii) or glomus tumors involving the infralabirintic space, auditory meatus, jugular bulb, and mastoid need an exposition that allows a more adequate vascular control and more significant bone resection to allow complete or near-total resection of the lesions. The patient should be ideally placed in the supine position, and his/her head should be rotated about 60–70 degrees contralaterally and fixed with the Mayfield head holder. Due to the need for a more considerable amount of autologous fat graft to later wound closure, we suggest the abdominal preparation for its eventual use. The incision follows from the anterior sternocleidomastoid (ECM) muscle to the retroauricular region, taking care to preserve the larger auricular nerve, since it may be a neural graft donor source for an eventual injury of intracranial nerves. Dissection of the neck allows adequate identification of the lower cranial nerves after their emergence of the skull, as well as carotid artery and internal jugular vein. Suboccipital craniectomy prior to mastoidectomy greatly facilitates control over the sigmoid sinus and jugular bulb, since the dura of the posterior fossa is less adhered to the sigmoid sinus and craniectomy facilitates its identification, reducing the possibility of injury to the sigmoid sinus even during mastoid air cells drilling. The cortical portion of the mastoid can be removed and used for the reconstruction of that region at the end of the procedure, and the mastoid air cells are drilled until a thin layer of bone remains over the sigmoid sinus/jugular vein's bulb. The venous structures are carefully separated, and the retrolabyrinthine bony portion resected until exposure of the posterior fossa dura. Care should be taken when drilling the anterior portion of this approach, avoiding entering the labyrinth and injuring the facial nerve. By drilling the intralabyrinthine portion, the extracranial portion of the tumor can be adequately resected. If there is an intracranial lesion, the opening of the dura mater in the presigmoid retrolabyrinthine region is followed, and the intradural resection is completed. In the case of preoperative anacusis (diagnosed by audiometry), presigmoid translabyrinthine approach, transcochlear approach and even posterior or total

petrosectomy can be performed to maximize resection (**Figures 4**–**9**).

*Skin incision (arrows on the left), muscle dissection and bur hole demarcations (on the right) for a posterior* 

#### *Jugular Foramen Paragangliomas DOI: http://dx.doi.org/10.5772/intechopen.84232*

*Brain and Spinal Tumors - Primary and Secondary*

are primarily intracranial located (Samii type A schwannomas) or tumors with a more significant intracranial extension may be approached by the classic lateral suboccipital retrosigmoid approach. The patient should be ideally positioned in a semi-sited position, since the presence of tumor bleeding gravitates downward, maintaining the surgical field with better visibility throughout the resection. Ideally, central venous access and pre-cordial Doppler should be used to prevent and treat air embolism. Pneumatic compression boots should be used to facilitate venous return. The head should be rotated about 30° to the same side of the lesion, aiming a straight direction in relation to the jugular foramen, as well as a smaller cerebellar retraction, and be fixed in a Mayfield head holder. Discrete flexion helps to expose the suboccipital region, facilitating the positioning of the surgeon during the approach. Vigorous rotation and flexion should be avoided as they may compromise jugular venous return; so a space of two fingers should separate the chin from the ipsilateral clavicle for this purpose. A cutaneous incision should be made with an upper limit on the pinna to the posterior musculature of the neck, maintaining a distance of about 3 cm from the mastoid. After incision of the muscular plane, suboccipital craniectomy is performed, ideally exposing the inferior portion of the transverse sinus and medial portion of the sigmoid sinus. The most crucial step of the craniectomy is its inferior extension to the posterior border of the magnum foramen. The dura mater should be cut in a "C" fashion with its convex portion of the cut close to the transverse and sigmoid sinuses. The dura mater, when cut in this way, not only protects the cerebellar hemisphere from contusions but also prevents dural redundancy in the visual field of

*Typical alveolar pattern with presence of Zellballen, which are classic epithelioid clusters of these tumors.*

the surgeon, also allowing more adequate closure at the end of the procedure.

Next, the arachnoid trabeculae of the Magna cistern of the pontocerebellar angle (PCA) should be cut, and careful aspiration of cerebrospinal fluid should follow, allowing relaxation of the neurovascular structures. Then, the anterior and medial portion of the cerebellum must be carefully covered with cottonoids and retracted medially and superiorly with a spatula, and then fixed in a static position. Dialogue with neurophysiologists is essential during this time of surgery, and repositioning of the spatula may be necessary in case of disturbed auditory brainstem evoked potential. It is essential to determine the exact position of the tumor in relation to the sigmoid sinus and bulb of the jugular vein, because in larger tumors of this region the wall of these vessels may be compromised, with catastrophic bleeding that is difficult to control. Extradural drilling of the jugular foramen helps to define the margins of the tumor, and after this maneuver follows the careful debulking of the tumor.

We carefully proceed with the dissection between the tumor and the lower cranial nerves, as well as its separation of the sigmoid sinus and bulb of the jugular vein when they are involved. The use of ultrasonic aspiration helps significantly during

**60**

**Figure 3.**

the resection of these tumors. As the tumor resection is performed, we maintain an incessant dialogue with the neurophysiologist observing the changes during the monitoring of the lower cranial nerves (IX, X, XI, XII). At the end of resection, hemostasis is followed, and defects in the skull base and mastoid should be covered with autologous fat and fibrin glue. The closure in subsequent layers is done, and extubation is monitored by an anesthesiologist in the operating room when there is no cranial nerve injury. Larger tumors (schwannomas B, C, and D of Samii) or glomus tumors involving the infralabirintic space, auditory meatus, jugular bulb, and mastoid need an exposition that allows a more adequate vascular control and more significant bone resection to allow complete or near-total resection of the lesions.

The patient should be ideally placed in the supine position, and his/her head should be rotated about 60–70 degrees contralaterally and fixed with the Mayfield head holder. Due to the need for a more considerable amount of autologous fat graft to later wound closure, we suggest the abdominal preparation for its eventual use. The incision follows from the anterior sternocleidomastoid (ECM) muscle to the retroauricular region, taking care to preserve the larger auricular nerve, since it may be a neural graft donor source for an eventual injury of intracranial nerves. Dissection of the neck allows adequate identification of the lower cranial nerves after their emergence of the skull, as well as carotid artery and internal jugular vein. Suboccipital craniectomy prior to mastoidectomy greatly facilitates control over the sigmoid sinus and jugular bulb, since the dura of the posterior fossa is less adhered to the sigmoid sinus and craniectomy facilitates its identification, reducing the possibility of injury to the sigmoid sinus even during mastoid air cells drilling. The cortical portion of the mastoid can be removed and used for the reconstruction of that region at the end of the procedure, and the mastoid air cells are drilled until a thin layer of bone remains over the sigmoid sinus/jugular vein's bulb. The venous structures are carefully separated, and the retrolabyrinthine bony portion resected until exposure of the posterior fossa dura. Care should be taken when drilling the anterior portion of this approach, avoiding entering the labyrinth and injuring the facial nerve. By drilling the intralabyrinthine portion, the extracranial portion of the tumor can be adequately resected. If there is an intracranial lesion, the opening of the dura mater in the presigmoid retrolabyrinthine region is followed, and the intradural resection is completed. In the case of preoperative anacusis (diagnosed by audiometry), presigmoid translabyrinthine approach, transcochlear approach and even posterior or total petrosectomy can be performed to maximize resection (**Figures 4**–**9**).

#### **Figure 4.**

*Skin incision (arrows on the left), muscle dissection and bur hole demarcations (on the right) for a posterior petrosal approach.*

#### **Figure 5.**

*Skin incision and bur hole demarcations for a posterior petrosal approach after the craniotomy (left side) and after mastoid drilling (right side).*

#### **Figure 6.**

*Presigmoid approach and tentorial incision (on the left). Three-dimensional perspective of the tumor and its relationship with neural, vascular and skull base structures (right side).*

#### **Figure 7.**

*On the left side, preoperative T1 weighted MRI with gadolinium from a patient operated in our institution; in the middle, preoperative embolization, 1 day before surgery. On the right side, artist's depiction of the tumor and its vascularity.*

**63**

*Jugular Foramen Paragangliomas*

**Figure 8.**

**Figure 9.**

*DOI: http://dx.doi.org/10.5772/intechopen.84232*

In cases of infiltration or occlusion of the sigmoid sinus by the tumor, its proximal and distal ligation can be performed, generally without the addition of deficits since the collateral venous drainage is developed by slow tumor growth. After hemostasis and verification of cranial nerve function by neurophysiologists, hemostasis follows. The retroauricular space with the mastoid should be filled with

*External auditory meatus invasion by the tumor on the left side and skin incision on the right side.*

Increasing evidence demonstrates that stereotactic radiosurgery, particularly Gamma-Knife (GK) surgery may play a relevant role in the treatment of these tumors. Results show no change in neurological signs and symptoms in up to 65% of patients [12]. Due to the best results with microsurgery, we prefer microsurgical

Possible complications include facial nerve damage, injury to the lower cranial nerves, injury to the internal carotid artery, excessive bleeding due to lesions of the

autologous fat graft and fibrin glue. Closure should than be performed.

*Approaches' visualization after craniotomy, partial incision of the tentorium.*

resection with the use of radiosurgery in residual or recurrent tumor.

**6. Treatment with radiotherapy**

**7. Postoperative complications and results**

*Brain and Spinal Tumors - Primary and Secondary*

*Skin incision and bur hole demarcations for a posterior petrosal approach after the craniotomy (left side) and* 

*Presigmoid approach and tentorial incision (on the left). Three-dimensional perspective of the tumor and its* 

*On the left side, preoperative T1 weighted MRI with gadolinium from a patient operated in our institution; in the middle, preoperative embolization, 1 day before surgery. On the right side, artist's depiction of the tumor* 

*relationship with neural, vascular and skull base structures (right side).*

**62**

**Figure 7.**

*and its vascularity.*

**Figure 5.**

**Figure 6.**

*after mastoid drilling (right side).*

**Figure 8.** *External auditory meatus invasion by the tumor on the left side and skin incision on the right side.*

**Figure 9.** *Approaches' visualization after craniotomy, partial incision of the tentorium.*

In cases of infiltration or occlusion of the sigmoid sinus by the tumor, its proximal and distal ligation can be performed, generally without the addition of deficits since the collateral venous drainage is developed by slow tumor growth. After hemostasis and verification of cranial nerve function by neurophysiologists, hemostasis follows. The retroauricular space with the mastoid should be filled with autologous fat graft and fibrin glue. Closure should than be performed.

## **6. Treatment with radiotherapy**

Increasing evidence demonstrates that stereotactic radiosurgery, particularly Gamma-Knife (GK) surgery may play a relevant role in the treatment of these tumors. Results show no change in neurological signs and symptoms in up to 65% of patients [12]. Due to the best results with microsurgery, we prefer microsurgical resection with the use of radiosurgery in residual or recurrent tumor.

## **7. Postoperative complications and results**

Possible complications include facial nerve damage, injury to the lower cranial nerves, injury to the internal carotid artery, excessive bleeding due to lesions of the venous structures (sigmoid sinus and internal jugular vein), and other complications such as cerebrospinal fluid fistula and infection. Larger tumors (C and D of Fisch) represent a greater surgical challenge, and cranial nerve deficits can be seen postoperatively and in around 6% of cases [13]. Facial paralysis can be seen in around 6% of cases, and cerebrospinal fluid fistula occurs in about 5% of cases [13]. Giant tumors with invasion of multiple structures have a more difficult but feasible resection, and malignant tumors have a reserved prognosis [14].

## **8. Conclusion**

Resection of paragangliomas is possible as long as accurate clinical evaluation and preoperative examinations are rigorously performed. Complications can occur during and after the surgery, and we must be adequately prepared for its treatment. The use of embolization in the preoperative period may considerably reduce bleeding during surgery, but it is not considered an innocuous procedure and may present cranial nerve paralysis due to vasa nervorum obstruction culminating with nerve ischemia. Once again we consider that experience is essential for its effective treatment.

## **Conflict of interest**

Authors declare no conflict of interest.

## **Author details**

Breno Nery1 \*, Rodrigo Antônio Fernandes Costa1 , Eduardo Quaggio1 , Ricardo Lopes Araújo2 , Bernardo Alves Barbosa2 , Diogo Fabricio Coelho de Melo2 , Carolina Salviano de Abreu Nery3 , Fred Bernardes Filho4 and George Peter Stevens<sup>5</sup>

1 Department of Neurosurgery, São Francisco Hospital, Ribeirão Preto, SP, Brazil

2 Department of Neurosurgery, Heliopolis Hospital, São Paulo, São Paulo, Brazil

3 Department of Radiology and Medical Imaging, Diagnóstico por Imagem de Alta Performance (DIAP), Ribeirão Preto, SP, Brazil

4 Department of Internal Medicine, São Francisco Hospital, Ribeirão Preto, SP, Brazil

5 College of Osteopathic Medicine, William Carey University, Hattiesburg, MS, USA

\*Address all correspondence to: brenonery84@gmail.com

© 2019 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

**65**

*Jugular Foramen Paragangliomas*

[1] Persky MS, Setton A, Niimi Y, Hartman J, Frank D, Berenstein A. Combined endovascular and surgical treatment of head and neck paragangliomas—A team approach. Head & Neck. 2002;**24**:423-431. DOI:

[2] Lee JH, Barich F, Karnell LH,

[3] Guinto G, Kageyama M, Trujillo-Luarca VH, Abdo M, Ruiz-Than A, Romero-Rangel A. Nonglomic tumors of the jugular foramen: Differential diagnosis and prognostic implications. World Neurosurgery. 2014;**82**:1283-1290. DOI: 10.1016/j.

[4] Nowak A, Dziedzic T, Czernicki T, Kunert P, Marchel A. Surgical treatment of jugular foramen meningiomas. Neurologia i neurochirurgia polska. 2014;**48**:391-396. DOI: 10.1016/j.

[5] Thomas AJ, Wiggins RH 3rd, Gurgel RK. Nonparaganglioma jugular foramen tumors. Otolaryngologic Clinics of North America. 2015;**48**:343-359. DOI:

[6] Jansen JC, van den Berg R, Kuiper A, van der Mey AG, Zwinderman AH, Cornelisse CJ. Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer. 2000;**88**:2811-2816. DOI: 10.1002/1097-

Robinson RA, Zhen WK, Gantz BJ, et al. National cancer data base report on malignant paragangliomas of the head and neck. Cancer. 2002;**94**:730-737.

10.1002/hed.10068

**References**

DOI: 10.1002/cncr.10252

wneu.2014.1207.1013

pjnns.2014.1009.1008

10.1016/j.otc.2014.1012.1008

0142(20000615)88:12<2811

10.1002/lary.1982.92.2.188

[7] Dickens WJ, Million RR, Cassisi NJ, Singleton GT. Chemodectomas arising in temporal bone structures. The Laryngoscope. 1982;**92**:188-191. DOI:

*DOI: http://dx.doi.org/10.5772/intechopen.84232*

[8] Gulya AJ. The glomus tumor and its biology. The Laryngoscope.

[9] Oldring D, Fisch U. Glomus tumors of the temporal region: Surgical therapy. The American Journal of Otology.

[10] Jackson CG, Glasscock ME 3rd, Harris PF. Glomus tumors. Diagnosis, classification, and management of large lesions. Archives of Otolaryngology.

[11] Samii M, Babu RP, Tatagiba M, Sepehrnia A. Surgical treatment of jugular foramen schwannomas. Journal of Neurosurgery. 1995;**82**:924-932. DOI:

[12] Gerosa M, Visca A, Rizzo P, Foroni R, Nicolato A, Bricolo A. Glomus jugulare tumors: The option of gamma knife radiosurgery. Neurosurgery. 2006;**59**:561-569. DOI: 10.1227/01. NEU.0000228682.92552.CA

10.3171/jns.1995.82.6.0924

[13] Makiese O, Chibbaro S,

Marsella M, Tran Ba Huy P, George B. Jugular foramen paragangliomas: Management, outcome and avoidance of complications in a series of 75 cases.

Neurosurgical Review. 2012;**35**: 185-194; discussion 194. DOI: 10.1007/

[14] Al-Mefty O, Teixeira A. Complex tumors of the glomus jugulare: Criteria, treatment, and outcome. Journal of Neurosurgery. 2002;**97**:1356-1366. DOI:

s10143-10011-10346-10141

10.3171/jns.2002.97.6.1356

1993;**103**:7-15

1979;**1**:7-18

1982;**108**:401-410

## **References**

*Brain and Spinal Tumors - Primary and Secondary*

**64**

Brazil

**Author details**

**8. Conclusion**

Ricardo Lopes Araújo2

**Conflict of interest**

Carolina Salviano de Abreu Nery3

Breno Nery1

treatment.

provided the original work is properly cited.

Performance (DIAP), Ribeirão Preto, SP, Brazil

Authors declare no conflict of interest.

\*Address all correspondence to: brenonery84@gmail.com

© 2019 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium,

5 College of Osteopathic Medicine, William Carey University, Hattiesburg, MS, USA

\*, Rodrigo Antônio Fernandes Costa1

, Bernardo Alves Barbosa2

, Fred Bernardes Filho4

1 Department of Neurosurgery, São Francisco Hospital, Ribeirão Preto, SP, Brazil

venous structures (sigmoid sinus and internal jugular vein), and other complications such as cerebrospinal fluid fistula and infection. Larger tumors (C and D of Fisch) represent a greater surgical challenge, and cranial nerve deficits can be seen postoperatively and in around 6% of cases [13]. Facial paralysis can be seen in around 6% of cases, and cerebrospinal fluid fistula occurs in about 5% of cases [13]. Giant tumors with invasion of multiple structures have a more difficult but feasible

Resection of paragangliomas is possible as long as accurate clinical evaluation and preoperative examinations are rigorously performed. Complications can occur during and after the surgery, and we must be adequately prepared for its treatment. The use of embolization in the preoperative period may considerably reduce bleeding during surgery, but it is not considered an innocuous procedure and may present cranial nerve paralysis due to vasa nervorum obstruction culminating with nerve ischemia. Once again we consider that experience is essential for its effective

resection, and malignant tumors have a reserved prognosis [14].

2 Department of Neurosurgery, Heliopolis Hospital, São Paulo, São Paulo, Brazil

4 Department of Internal Medicine, São Francisco Hospital, Ribeirão Preto, SP,

3 Department of Radiology and Medical Imaging, Diagnóstico por Imagem de Alta

, Eduardo Quaggio1

,

and George Peter Stevens<sup>5</sup>

,

, Diogo Fabricio Coelho de Melo2

[1] Persky MS, Setton A, Niimi Y, Hartman J, Frank D, Berenstein A. Combined endovascular and surgical treatment of head and neck paragangliomas—A team approach. Head & Neck. 2002;**24**:423-431. DOI: 10.1002/hed.10068

[2] Lee JH, Barich F, Karnell LH, Robinson RA, Zhen WK, Gantz BJ, et al. National cancer data base report on malignant paragangliomas of the head and neck. Cancer. 2002;**94**:730-737. DOI: 10.1002/cncr.10252

[3] Guinto G, Kageyama M, Trujillo-Luarca VH, Abdo M, Ruiz-Than A, Romero-Rangel A. Nonglomic tumors of the jugular foramen: Differential diagnosis and prognostic implications. World Neurosurgery. 2014;**82**:1283-1290. DOI: 10.1016/j. wneu.2014.1207.1013

[4] Nowak A, Dziedzic T, Czernicki T, Kunert P, Marchel A. Surgical treatment of jugular foramen meningiomas. Neurologia i neurochirurgia polska. 2014;**48**:391-396. DOI: 10.1016/j. pjnns.2014.1009.1008

[5] Thomas AJ, Wiggins RH 3rd, Gurgel RK. Nonparaganglioma jugular foramen tumors. Otolaryngologic Clinics of North America. 2015;**48**:343-359. DOI: 10.1016/j.otc.2014.1012.1008

[6] Jansen JC, van den Berg R, Kuiper A, van der Mey AG, Zwinderman AH, Cornelisse CJ. Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer. 2000;**88**:2811-2816. DOI: 10.1002/1097- 0142(20000615)88:12<2811

[7] Dickens WJ, Million RR, Cassisi NJ, Singleton GT. Chemodectomas arising in temporal bone structures. The Laryngoscope. 1982;**92**:188-191. DOI: 10.1002/lary.1982.92.2.188

[8] Gulya AJ. The glomus tumor and its biology. The Laryngoscope. 1993;**103**:7-15

[9] Oldring D, Fisch U. Glomus tumors of the temporal region: Surgical therapy. The American Journal of Otology. 1979;**1**:7-18

[10] Jackson CG, Glasscock ME 3rd, Harris PF. Glomus tumors. Diagnosis, classification, and management of large lesions. Archives of Otolaryngology. 1982;**108**:401-410

[11] Samii M, Babu RP, Tatagiba M, Sepehrnia A. Surgical treatment of jugular foramen schwannomas. Journal of Neurosurgery. 1995;**82**:924-932. DOI: 10.3171/jns.1995.82.6.0924

[12] Gerosa M, Visca A, Rizzo P, Foroni R, Nicolato A, Bricolo A. Glomus jugulare tumors: The option of gamma knife radiosurgery. Neurosurgery. 2006;**59**:561-569. DOI: 10.1227/01. NEU.0000228682.92552.CA

[13] Makiese O, Chibbaro S, Marsella M, Tran Ba Huy P, George B. Jugular foramen paragangliomas: Management, outcome and avoidance of complications in a series of 75 cases. Neurosurgical Review. 2012;**35**: 185-194; discussion 194. DOI: 10.1007/ s10143-10011-10346-10141

[14] Al-Mefty O, Teixeira A. Complex tumors of the glomus jugulare: Criteria, treatment, and outcome. Journal of Neurosurgery. 2002;**97**:1356-1366. DOI: 10.3171/jns.2002.97.6.1356

**67**

Section 4

Surgical Approaches to

CNS Tumors

## Section 4
