*10.1.2 Endoscopic approach*

Last decade has seen a paradigm shift from open approach to transnasal endoscopic approach. In today's time, endoscopic surgery can be regarded as the most rapidly advancing surgical field. As the surgeon's familiarity with the endoscopes is increasing, hard to reach anatomical regions are also becoming more accessible, thereby, widening the horizon for this approach. Tumors, which were earlier labeled as operable via an open approach only, can now be easily and completely resected using endoscopic approach.

Endoscopic surgery has the advantage of better illumination and magnification, lower morbidity, and shorter duration of hospital stay which ultimately leads to cost saving. Advantage of no visible facial scar adds to the cosmetic viability of this approach.

## *10.1.2.1 Surgical considerations for endoscopic jna surgery*

1.Tumor size and extent decides the exact endoscopic approach required. While smaller tumors are managed via an endonasal approach; medium to large sized tumors require an endoscopic Denker's / Sturman- Canfield or a more extensive transpterygoid approach [57, 58].

Extended anterior skull base approaches are recommended for intracranial lesions [59].


*Juvenile Nasopharyngeal Angiofibroma DOI: http://dx.doi.org/10.5772/intechopen.95923*

**Figure 4.** *CE-MRI showing hyperintense tumor (\*) in the infratemporal fossa.*

#### **Figure 5.**

*CECT showing JNA occupying nasopharynx (1), pterygoid wedge (2), infratemporal fossa (3) and intracranial space (4). Notice the widening of left pterygoid wedge (red arrow) as compared to the right normal pterygoid wedge (green arrow)-* Ram Haran Sign*.*

*10.1.2.2 Contraindications to endoscopic approach*


Considering the pace of progress in endoscopic techniques, it would not be surprising if some more indications are added by the time this chapter reaches the readers.

#### **Figure 6.**

*CT angiography showing a vascular tumor (\*). Notice the internal maxillary artery supplying this tumor (orange arrow).*

#### **Figure 7.**

*Endoscopic view through left nasal cavity: Medial and posterior walls of left maxillary sinus and left inferior turbinate have been removed. 1-nasopharyngeal component of JNA; 2- pterygopalatine fossa + infratemporal fossa component of JNA; 3- remnants of left inferior turbinate; R- right, L- left, S- superior, I- inferior.*

#### **10.2 Non-surgical treatment of JNA: adjuvant treatment modalities**

Though Juvenile angiofibroma is now an established surgical entity, there has been an era when medical management alone was the rule for extensive tumors especially those with intracranial extension. With paradigm shift towards more aggressive surgical procedures for all stages of the tumor, other treatment modalities are now valued as adjuvant therapy only.

#### *10.2.1 Embolization*

**Transarterial embolization (TAE)** is done preoperatively to decrease the blood flow to the tumor, thereby, reducing the intraoperative blood loss and need for blood transfusion. This is particularly useful for tumors with advanced stage.

#### **Figure 8.**

*Coblation wand being used in juvenile nasopharyngeal angiofibroma endoscopic surgery. 1- coblation wand, 2 tumor, R- right, L- left, S- superior, I- inferior.*

Smaller tumors have less vascularity and can be resected easily even without preoperative embolization [61].

The procedure is usually done 24 to 48 hours before the scheduled surgery. Further surgical delay is not appreciated/recommended as tumor gains collateral blood supply through neoangiogenesis. A wide variety of materials are available as embolic agents: microspheres, gelatin sponge, Teflon particles, gel foam, poly-vinyl alcohol, polystyrene, silicone particles, silk, cyanoacrylate, sodium tetradacyl sulphate, autogenous clot, duramater, muscle fragments, etc. 300–500 micrometer spheres are preferred owing to greater blocking capacity of vascular lumen [62].

The procedure is not without complications. Cerebral ischemia and vision loss are known complications following embolic agent migrating to ICA system. Rare complications like cerebral edema, hemiplegia and aphasia have also been reported [63].

**Direct Puncture Therapeutic Embolization (DPTE)** is a new concept for tumor embolization. Embolizing agent is a mixture of n-butyl cyanoacrylate [NBCA], lipiodol, powdered tungsten with/without absolute ethanol. Under fluoroscopic visualization, embolizing agent is injected directly into the tumor through a percutaneous route or a transoral/ transnasal/transpalatal route [64].

This results in almost complete filling of tumor microvasculature with irreversible occlusion of embolized vessels. Tumor gains a dark color (due to tungsten powder with blue dye) which helps to better distinguish it from surrounding normal tissue. Direct cytotoxicity of absolute ethanol has shown good therapeutic effects.

DPTE alone or in combination with TAE has shown to have better devascularisation effects than TAE alone [65, 66].

#### *10.2.2 Hormonal therapy*

Hormonal influence on growth of JNA has been speculated since long. An interplay between estrogens and androgens has been associated with tumor proliferation and its spontaneous involution. Various hormonal therapies are recommended based on these concepts.

**Estrogen therapy:** exogenous estrogen has been tried traditionally with the aim of decreasing tumor size and vascularity. Lack of conclusive therapeutic advantage, feminizing side effects and propensity towards cardiovascular side effects have rendered its place to be of historical significance only.

**Anti-androgen therapy:** Flutamide is a non-steroidal androgen receptor blocker drug, primarily used in prostatic cancer. It binds with the androgen receptors, thereby blocking the action of testosterone. Recently, it has been proven that the response to flutamide therapy is much more pronounced in post-pubertal patients as compared to pre-pubertal patients [23].

Flutamide therapy is recommended as a six week preoperative adjuvant therapy for intracranial and intraorbital lesions, recurrent lesions and those with their blood supply primarily from ICA.

#### *10.2.3 Radiotherapy*

Low dose radiotherapy is used for angiofibromas extending intracranially, not amenable to primary surgery. Typically, total radiation dose of 3,500 cGy is given over 3 weeks. A successful response in terms of decreased tumor size and vascularity is seen over several months in 80% of the patients [67, 68]. Those showing no response/incomplete response by 2 years post radiotherapy are deemed as failures and taken up for salvage surgery.

There are numerous side effects to use of radiotherapy at a young age. Posterior capsular opacities, glaucoma, optic nerve atrophy, xerostomia, hypopituitarism, cerebral necrosis, osteoradionecrosis of mandible, skull base osteomyelitis, risk of developing new head–neck tumors later in life, potential malignant transformation of angiofibromas are few of the complications associated with the use of radiotherapy in head and neck region.

**Intensity Modulated Radiotherapy (IMRT)** allows higher doses to be given to the lesion without damaging adjoining normal tissues. Multiple beams from different directions converge onto the tumor shape so that the target area has the highest dose strength with relative sparing of surrounding vital structures.

**Gamma Knife** makes use of radiation beams from 201 sources, converging onto a single point. This causes retardation of further tumor growth. **Cyber Knife** is a type of stereotactic radiosurgery which uses a robotic arm to deliver radiations to a point source. These are being applied in association with other treatment modalities to achieve desired results in large angiofibromas [69, 70].

#### **11. Conclusion**

Juvenile nasopharyngeal angiofibroma, although an old disease entity, is still fascinating medical experts all over the world. Although still largely unknown, with advanced genetic and molecular studies, we have moved a step closer to find the origin and etiology of this disease. At present, surgery is the mainstay of treatment with endoscopic approach replacing the conventional open approach. Future considerations can be focused on therapeutic embolisation, stereotactic radiotherapy and targeted molecular therapy for a non-surgical cure.

#### **Acknowledgements**

I wish to express my sincere gratitude towards Dr. Anupama Mahajan, Dr. Stuti Mahajan and Dr. Anugeet Sethi for their constant moral support. I am fortunate to

*Juvenile Nasopharyngeal Angiofibroma DOI: http://dx.doi.org/10.5772/intechopen.95923*

have worked with Dr. Rajesh Choudhary and Dr. Bikramjeet Singh on numerous juvenile nasopharyngeal angiofibroma cases. A special vote of thanks to Dr. Rohan Sardana and Dr. Karamjeet Singh Gill for providing valuable insight into the pathology of this disease. I shall always be indebted to ENT Department of my alma mater VMMC & Safdarjung Hospital, New Delhi.
