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nasopharyngeal carcinoma (NPC)

Etiology: - environmental: consumption of preserved foods with volatile nitrosamines eg. Cantonese style salted fish - association with latent Epstein Barr virus infection Epidemiology: - type III (undifferentiated carcinoma) more prevalent (~97%) in endemic areas such as southern China - type I (keratinizing squamous cell carcinoma) more common in western countries - latent Epstein Barr virus infection uniquely present in almost all NPC from endemic regions Pathology: WHO classification -> type I: keratinizing squamous cell carcinoma -> type II: non-keratinizing squamous cell carcinoma -> type III: undifferentiated carcinoma - lymphoplasmacytic infiltrates typically prominant Genetics: - associated with HLA-A2 subtype HLA-A*0207 - frequent loss of chromosomes 3p, 9, 11q, 13q, 14q, 16q - recurrent gains of chromosome 12 - gene alterations - p16/CDNK2A hypermethylation/ 62-86% homozygous deletion - p14/ARF hypermethylation/ 54% homozygous deletion - p63 overexpression 100% - bcl-2 overexpression 80% - bcl-3 overexpression 60% - DAP kinase hypermethylation 76% - EGFR overexpression 85% - E-cadherin hypermethylation 52% - TSLC1 hypermethylation 34% - RASSF1A hypermethylation/mutation 67-83% - RARB2 hypermethylation 80% - OSCP1 - other implicated genes: NGX6, TP53, ACOT7L Clinical manifestations: - neck mass/cervical lymphadenopathy - blood tinged sputum - nasal obstruction - increasing nasal discharge - tinnitus - conductive hearing loss - headache (trigeminal neuralgia) - immobility of ipsilateral soft palate Management: 1) radiation therapy for stage I disease a) tumors are highly radiosensitive b) lymph node dissection NOT necessary 2) radiation with adjuvant chemotherapy for more advanced disease 3) chemotherapy has no effect on overall survival

Related

head & neck cancer (HNC)

Specific

nasal carcinoma

General

squamous cell carcinoma (SCC) nasopharyngeal neoplasm

Database Correlations

OMIM correlations

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 17. American College of Physicians, Philadelphia 1998
  2. Lo KW et al. Focus on nasopharyngeal carcinoma. Cancer Cell 5:423-8, 2004 PMID: 15144950