Presentation on theme: "Nasopharyngeal Angiofibroma: l Juvenile Nasopharyngeal Angiofibroma (JNA) l highly vascular benign yet unencapsulated tumor of adolescent males."— Presentation transcript:
Nasopharyngeal Angiofibroma: l Juvenile Nasopharyngeal Angiofibroma (JNA) l highly vascular benign yet unencapsulated tumor of adolescent males.
l Frequency: JNA accounts for 0.05% of all head and neck tumors. l Sex: JNA occurs exclusively in males. l Age: range is 7-19 years. JNA is rare in patients older than 25 years l Etiology: A hormonal theory has been suggested due to the lesion's occurrence in adolescent males.
l 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.
l Clinical: Symptoms: l Nasal obstruction (80-90%): l Epistaxis (45-60%): l Headache (25%): l Facial swelling (10-18%) l Other symptoms include unilateral rhinorrhea, anosmia, hyposmia, rhinolalia, deafness, otalgia, swelling of the palate, and deformity of the cheek.
Signs: l Nasal mass (80%) l Orbital mass (15%) l Proptosis (10-15%) l 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: l Other causes of nasal obstruction, (eg, nasal polyps, antrochoanal polyp, teratoma, encephalocele, dermoids, inverting papilloma, rhabdomyosarcoma, squamous cell carcinoma) l Other causes of epistaxis, systemic or local l Other causes of proptosis or orbital swelling
Medical therapy: l Surgical therapy l Hormonal therapy l Radiotherapy Biopsy is prohibited because of severe bleeding
Anatomy l Anteriorly -- nasal cavity l Posteriorly -- skull base and vertebral bodies l Inferiorly -- oropharynx and soft palate l Laterally -- –Eustachian tubes and tori –Fossa of Rosenmuller - most common location
Anatomy l Close association with skull base foramen l Mucosa –Epithelium - tissue of origin of NPC Stratified squamous epithelium Pseudostratified columnar epithelium –Salivary, Lymphoid structures
Epidemiology l Chinese native ( 广东，广西。湖南，福建） l > Chinese immigrant > North American nativeBoth genetic and environmental factors l Genetic –HLA histocompatibility loci possible markers
Epidemiology l 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 l 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 l Xerophthalmia - greater sup. petrosal n l Facial pain - Trigeminal n. l Diplopia - CN VI l Ophthalmoplegia - CN III, IV, and VI –cavernous sinus or superior orbital fissure l Horner’s syndrome - cervical sympathetics l CN’s IX, X, XI, XII - extensive skull base
Clinical Presentation l Nasopharyngeal examination –Fossa of Rosenmuller most common location
l Regional spread –Usually ipsilateral first but bilateral not uncommon l Distant spread - rare (<3%), lungs, liver, bones
Radiological evaluation l Contrast CT with bone and soft tissue windows –imaging tool of choice for NPC l MRI –soft tissue involvement, recurrences l Chest CT, bone scans
l TNM classification l Class 0: T is N 0 M 0 l Class I: T 1 N 0 M 0 l Class II A: T 2a N 0 M 0 l Class II B: T 1 N 1 M 0 ; T 2a N 1 M 0 ; T 2b N 0, N 1 M 0 l Class III: T 1 N 2 M 0 ; T 2a, T 2b N 1 M 0 ; T 3 N 0, N 1, N 2 M 0
l Class IV A: T 4 N 0, N 1, N 2 M 0 l Class IV B: any T N 3 M 0 l Class IV C: any T any N M 1
Treatment l External beam radiation –Dose: cGy l Adjuvant brachytherapy –mainly for residual/recurrent disease
Treatment Surgical management l Mainly diagnostic - Biopsy l Surgical treatment –primary lesion –regional failure with local control
Treatment Surgical management l Primary lesion –consider for residual or recurrent disease –approaches infratemporal fossa transparotid temporal bone approach transmaxillary transmandibular transpalatal
Treatment Surgical management l Regional disease –Neck dissection may offer improved survival compared to repeat radiation of the neck
Treatment l Chemotherapy l Immunotherapy
Congenital tonsillar masses l Teratoma l Hemangioma l Lymphangioma l Cystic hygroma
l Treatment: l Laser therapy l Surgery
Malignant Neoplasms l Most common is lymphoma l Non-Hodgkin’s lymphoma l Rapid unilateral tonsillar enlargement associated with cervical lymphadenopathy and systemic symptoms
l Treatment l Radiotherapy and chemical therapy