Nasopharyngeal carcinoma
Nasopharyngeal carcinoma
Updated: 10/13/2020
© Jun Wang, MD, PhD
General features
- Arising from the epithelium of the nasopharynx
- Common in South Asia, North Africa, Middle East and the Arctic
- More common in men
- Rare in the states, but incident increasing in black teenagers
- Bimodal age distribution, a small peak in late childhood, and second peak around 50-59
Risk factors
- Associated with Epstein-Barr virus infection
- Other risk factors: Consumption of salt-preserved fish containing carcinogenic nitrosamines, family history, specific HLA class I genotypes, tobacco smoking, chronic respiratory tract conditions and low consumption of fresh fruits and vegetables
Pathogenesis
- EBV genome products (LMP1, LMP2, etc) associated telomerase dysregulation, bcl2 overexpression, etc.
- Loss of function mutation of NF-kappa B inhibitors (CYLD, TRAF3, etc)
- Multiple chromosomal abnormalities
- p16 deletion
- LTBR (lymphotoxin-beta receptor) amplification
Clinical presentations
- Most common symptoms: Headache, diplopia, or facial numbness
- Clinical triad: Neck mass, nasal obstruction with epistaxis, serous otitis media
- Often bilateral neck involvement
- Cranial nerve palsy
- Nasopharynx mass by nasopharyngoscopy
Key morphological features
- Three histologic subtypes:
Keratinizing
squamous cell carcinoma (WHO type 1): Invasive carcinoma with obvious squamous differentiation
Nonkeratinizing-differentiated
(WHO type 2): Cords or trabeculae, with little or no keratinization
Nonkeratinizing-undifferentiated
(WHO type 3): Most common type, cohesive cells with indistinct cell margins
or diffuse cellular infiltrate of non-cohesive cells resembling non-Hodgkin
lymphoma
Marker
- Positive for cytokeratin, EMA
- Positive for EBV antigen, EBER (EBV encoded RNA)
- Negative for lymphocytic markers: CD45 etc
Diagnosis
- EBV studies: IgG against early EBV antigen; IgA against viral capsid antigen
- Radiologic studies
- Blind biopsies, particularly in fossa of Rosenmuller
Differential diagnosis:
- Nasal Polyps: Microscopic morphology
- Pediatric Non-Hodgkin Lymphoma: Positive for lymphocytic markers
- Pediatric Rhabdomyosarcoma: Positive for desmin, negative for cytokeratin
Treatment
- Radiation therapy: Mainstay of treatment
- Chemotherapy for advanced cases
- Usually unresectable by surgery
Poor prognostic factors
- High staging
- Keratinized type
- Metastasis
- Older age
- High plasma/serum EBV DNA titers
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