Nasopharyngeal Angiofibroma: l Juvenile Nasopharyngeal Angiofibroma (JNA) l highly vascular benign yet unencapsulated tumor of adolescent males.

Nasopharyngeal

Angiofibroma:

Juvenile Nasopharyngeal Angiofibroma

(JNA) highly vascular benign yet

unencapsulated tumor of adolescent males.

Frequency: JNA accounts for 0.05% of all head and neck tumors.

Sex: JNA occurs exclusively in males.

Age: range is 7-19 years. JNA is rare in patients older than 25 years

Etiology: A hormonal theory has been suggested due to the lesion's occurrence in adolescent males.

Pathophysiology:

The tumor starts adjacent to the sphenopalatine foramen. Large tumors frequently are bilobed or dumbbell-shaped, with one portion of the tumor filling the nasopharynx and the other portion extending to the pterygopalatine fossa.

Clinical: Symptoms: Nasal obstruction (80-90%): Epistaxis (45-60%): Headache (25%): Facial swelling (10-18%) Other symptoms include unilateral

rhinorrhea, anosmia, hyposmia, rhinolalia, deafness, otalgia, swelling of the palate, and deformity of the cheek.

Signs:

Nasal mass (80%)

Orbital mass (15%)

Proptosis (10-15%)

Other signs may include -Serous otitis due to eustachian tube blockage. -Zygomatic swelling and trismus denote spread of the tumor

to the infratemporal fossa.

Differentials:

Other causes of nasal obstruction, (eg, nasal polyps, antrochoanal polyp, teratoma, encephalocele, dermoids, inverting papilloma, rhabdomyosarcoma, squamous cell carcinoma)

Other causes of epistaxis, systemic or local

Other causes of proptosis or orbital swelling

CT scan

Medical therapy:

Surgical therapy Hormonal therapy Radiotherapy

Biopsy is prohibited because of severe bleeding

Nasopharyngeal malignancies

– Nasopharyngeal carcinoma (NPC)– Lymphoma– Salivary gland tumors– Sarcomas

Nasopharyngeal

Carcinoma

More common in Asia

Anatomy

Anteriorly -- nasal cavity Posteriorly -- skull base and vertebral

bodies Inferiorly -- oropharynx and soft palate Laterally --

– Eustachian tubes and tori– Fossa of Rosenmuller - most common location

Anatomy

Close association with skull base foramen Mucosa

– Epithelium - tissue of origin of NPC• Stratified squamous epithelium

• Pseudostratified columnar epithelium

– Salivary, Lymphoid structures

Epidemiology

Chinese native (广东，广西。湖南，福建） > Chinese immigrant > North American

nativeBoth genetic and environmental factors

Genetic– HLA histocompatibility loci possible markers

Epidemiology

Environmental– Viruses

• EBV- well documented viral “fingerprints” in tumor cells and also anti-EBV serologies with WHO type II and III NPC

• HPV - possible factor in WHO type I lesions

– Nitrosamines - salted fish

– Others - chronic nasal infection, poor hygiene, poor ventilation

Clinical Presentation

Often subtle initial symptoms1. unilateral HL (SOM)

2. epistaxis

3. painless, slowly enlarging neck mass

Larger lesions

4. nasal obstruction

5. cranial nerve involvement

Clinical Presentation

Xerophthalmia - greater sup. petrosal n Facial pain - Trigeminal n. Diplopia - CN VI Ophthalmoplegia - CN III, IV, and VI

– cavernous sinus or superior orbital fissure Horner’s syndrome - cervical sympathetics CN’s IX, X, XI, XII - extensive skull base

Clinical Presentation

Nasopharyngeal examination

– Fossa of Rosenmuller most common location

Regional spread– Usually ipsilateral first but bilateral not

uncommon

Distant spread - rare (<3%), lungs, liver, bones

Radiological evaluation

Contrast CT with bone and soft tissue windows– imaging tool of choice for NPC

MRI– soft tissue involvement, recurrences

Chest CT, bone scans

TNM classification Class 0: Tis N0 M0

Class I: T1 N0 M0

Class II A: T2a N0 M0

Class II B: T1 N1 M0 ; T2a N1 M0 ; T2b N0, N1 M0

Class III: T1 N2 M0 ; T2a, T2b N1 M0 ;

T3 N0, N1, N2 M0

Class IV A: T4 N0, N1, N2 M0

Class IV B: any T N3 M0

Class IV C: any T any N M1

Treatment

External beam radiation– Dose: 6500-7000 cGy

Adjuvant brachytherapy– mainly for residual/recurrent disease

Treatment Surgical management

Mainly diagnostic - Biopsy

Surgical treatment– primary lesion – regional failure with local control

Treatment Surgical management

Primary lesion – consider for residual or recurrent disease– approaches

• infratemporal fossa

• transparotid temporal bone approach

• transmaxillary

• transmandibular

• transpalatal

Treatment Surgical management

Regional disease– Neck dissection may offer improved survival

compared to repeat radiation of the neck

Treatment

Chemotherapy

Immunotherapy

Congenital tonsillar masses

Teratoma Hemangioma Lymphangioma Cystic hygroma

Treatment:

Laser therapy Surgery

Malignant Neoplasms

Most common is lymphoma Non-Hodgkin’s lymphoma Rapid unilateral tonsillar enlargement

associated with cervical lymphadenopathy and systemic symptoms

Treatment

Radiotherapy and chemical therapy