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Nasopharyngeal Carcinoma

Nasopharyngeal Carcinoma. Introduction. It is prevalent in Southern China, Southeast Asia, HongKong and parts of East and North Africa. High index of suspicion required for early diagnosis. Anatomy. Anteriorly -- nasal cavity Posteriorly -- skull base and vertebral bodies

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Nasopharyngeal Carcinoma

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  1. Nasopharyngeal Carcinoma

  2. Introduction • It is prevalent in Southern China,Southeast Asia, HongKong and parts of East and North Africa. • High index of suspicion required for early diagnosis

  3. 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

  4. Anatomy • Close association with skull base foramen • Mucosa • Epithelium - tissue of origin of NPC • Stratified squamous epithelium • Pseudostratified columnar epithelium • Salivary, Lymphoid structures

  5. Epidemiology • Chinese native > Chinese immigrant > North American native • Both genetic and environmental factors • Genetic • HLA histocompatibility loci possible markers

  6. Epidemiology • Environmental • Viruses • EBV- well documented viral “fingerprints” in tumor cells and also anti-EBV serologies • Nitrosamines - salted fish • Others - polycyclic hydrocarbons, chronic nasal infection, poor hygiene, poor ventilation

  7. Classification • WHO classes • Based on light microscopy findings • Type I - “SCCA” • 25 % of NPC • moderate to well differentiated cells similar to other SCCA ( keratin, intercellular bridges)

  8. Classification • Type II - “non-keratinizing” carcinoma • 12 % of NPC • variable differentiation of cells ( mature to anaplastic) • minimal if any keratin production • may resemble transitional cell carcinoma of the bladder

  9. Classification • Type III - “undifferentiated” carcinoma • 60 % of NPC, majority of NPC in young patients • Difficult to differentiate from lymphoma by light microscopy requiring special stains & markers • Diverse group • Lymphoepitheliomas, spindle cell, clear cell and anaplastic variants

  10. Classification • Differences between type I and types II & III • 5 year survival • Type I - 10% Types II, III - 50% • Long-term risk of recurrence for types II & III • Viral associations • Type I - HPV • Types II, III - EBV

  11. Clinical Presentation • Often subtle initial symptoms • unilateral hearing loss (SOM) • painless, slowly enlarging neck mass • Larger lesions • nasal obstruction • epistaxis • cranial nerve involvement

  12. 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

  13. Clinical Presentation • Nasopharyngeal examination • Fossa of Rosenmuller most common location • Variable appearance - exophytic, submucosal • Regional spread • Usually ipsilateral first but bilateral not uncommon • Distant spread - rare

  14. Diagnose • Biopsy under naso-endoscopy — Gold standard — sometimes repeated biopsy is needed — additional immunohistochemistry

  15. 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

  16. Nasopharyngeal carcinoma. A: Axial contrast-enhanced computed tomography (CECT) demonstrates enhancing lesion (asterisk) involving the pharyngeal mucosa space, retropharyngeal spaces, and prevertebral space. A tumor abuts the skull base. B: Axial CECT image with bone settings at the level of the skull base demonstrates a lytic destructive lesion involving the anteromedial left petrous bone (asterisk), medial portion of greater sphenoid wing (arrowhead), and adjacent clivus (arrow).

  17. Laboratory evaluation • Special diagnostic tests • IgA antibodies for viral capsid antigen (VCA) very popular in China • IgG antibodies for early antigen (EA)

  18. Staging • Variety of systems used • Am Jt Comm for Ca Staging • International Union Against Ca • Ho System • Unique NPC prognostic factors often not considered and similar prognosis between stages

  19. Treatment • External beam radiation: first choice • Dose: 6500-7000 cGy • Primary, upper cervical nodes, pos. lower nodes • Consider 5000 cGy prophylactic tx of clinically negative lower neck • Adjuvant brachytherapy • mainly for residual/recurrent disease

  20. Treatment • External beam radiation - complications • Include • xerostomia, tooth decay • ETD - early (SOM), later (patulous ET) • Endocrine disorders - hypopituitarism, hypothyroidism, hypothalamic disfunction • Soft tissue fibrosis including trismus • Ophthalmologic problems • Skull base necrosis

  21. TreatmentSurgical management • Primary lesion • consider for residual or recurrent disease • approaches • infratemporal fossa • transparotid temporal bone approach • transmaxillary • transmandibular • transpalatal

  22. TreatmentSurgical management • Regional disease • Neck dissection may offer improved survival compared to repeat radiation of the neck

  23. Treatment • Chemotherapy • Variety of agents • Chemotherapy + XRT - no proven long term benefit • Mainly for palliation of distant disease • Immunotherapy • Future treatment?? • Vaccine??

  24. Conclusion • Prevalent in Southern China, Southeast Asia, HongKong. And rare in North America, and Europe • Biopsy is the gold standard for diagnosis. • Treatment is primarily Radiation, not surgery.

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