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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