nasopharyngeal carcinoma (NPC)
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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
- radiation therapy for stage I disease
- tumors are highly radiosensitive
- lymph node dissection NOT necessary
- radiation with adjuvant chemotherapy for more advanced disease
- chemotherapy has no effect on overall survival
More general terms
More specific terms
Additional terms
References
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, 17. American College of Physicians, Philadelphia 1998
- ↑ Lo KW et al. Focus on nasopharyngeal carcinoma. Cancer Cell 5:423-8, 2004 PMID: https://www.ncbi.nlm.nih.gov/pubmed/15144950