nasopharyngeal angiofibroma: l juvenile nasopharyngeal angiofibroma (jna) l highly vascular benign...
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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
Medical therapy:
Surgical therapy Hormonal therapy Radiotherapy
Biopsy is prohibited because of severe bleeding
Nasopharyngeal malignancies
– Nasopharyngeal carcinoma (NPC)– Lymphoma– Salivary gland tumors– Sarcomas
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
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
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
Malignant Neoplasms
Most common is lymphoma Non-Hodgkin’s lymphoma Rapid unilateral tonsillar enlargement
associated with cervical lymphadenopathy and systemic symptoms