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 Nitrosamines - salted fish 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 symptoms 1. 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

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