Nasopharyngeal carcinoma

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Nasopharyngeal carcinoma, abbreviated NPC, is malignant epithelial tumour of the head and neck closely related to squamous cell carcinoma.

Nasopharyngeal carcinoma
Diagnosis in short

Nasopharyngeal carcinoma. H&E stain.

Synonyms nasopharyngeal-type undifferentiated carcinoma, non-keratining nasopharyngeal carcinoma undifferentiated type

LM Cohesive typical (squamoid) cells (abundant dense eosinophilic cytoplasm, central nuclei +/- small/indistinct nucleoli), prominent lymphoid component
Subtypes keratinizing SCC; nonkeratinizing carcinoma, differentiated; nonkeratinizing carcinoma, undifferentiated; basaloid SCC
LM DDx HPV-associated head and neck squamous cell carcinoma, squamous cell carcinoma of the head and neck
IHC p16 -ve, LMP-1 -ve/+ve
Molecular EBER +ve
Site nasopharynx - see head and neck

Signs neck mass
Prevalence uncommon in West

General

  • "Nasopharyngeal carcinoma" is the name of an entity - it is not a descriptive term.
  • Strong association with Epstein-Barr virus (EBV).
  • Moderate risk increase for smokers.[1]
  • Uncommon in the Western World[2] - endemic in the East.

Clinical:

  • Neck mass - most common presentation.[2]

Note:

Gross

  • Nasopharynx - as the name would suggest.
    • Nasal cavity involvement - common in early disease.[3]

Microscopic

Features:[4]

  • Prominent lymphoid component - key feature.
  • Features of squamous cell carcinoma:
    • Cohesive cells with:
      • Abundant dense eosinophilic cytoplasm.
      • Central nuclei +/- small/indistinct nucleoli.

Note:

  • May be keratinizing or nonkeratinizing[5] and the distinction is important - see classification below.

DDx:

Images

Case 1

Case 2

www

Histologic subclassification

World Health Classification (2005) for NPC:[6]

Type Histology Description EBV Prevalence Prognosis
1 keratinizing SCC graded poorly-well-diff. -ve ? bad
2a nonkeratinizing carcinoma, differentiated well def. cell borders & tumour nest borders, mimics appearance of UCC +ve ? good
2b nonkeratinizing carcinoma, undifferentiated sheets/syncytial, vescicular nuclei, prominent nucleoli, pink cytoplasm ? most common ?
3 basaloid SCC mimics BCC - see basaloid SCC ? least common ?

How to remember KNUB:

  • Keratinizing, Non-keratinizing diff., non-keratinizing Undiff., Basaloid SCC.

IHC

  • p16 -ve.[7]
  • LMP-1 (EBV-LMP) +ve/-ve.
    • Not sensitive; only positive in 40-45% of cases in a series of 30 cases.[8]

Notes:

Molecular

Images

See also

References

  1. Xue, WQ.; Qin, HD.; Ruan, HL.; Shugart, YY.; Jia, WH. (Aug 2013). "Quantitative association of tobacco smoking with the risk of nasopharyngeal carcinoma: a comprehensive meta-analysis of studies conducted between 1979 and 2011.". Am J Epidemiol 178 (3): 325-38. doi:10.1093/aje/kws479. PMID 23785114.
  2. 2.0 2.1 Colaco, RJ.; Betts, G.; Donne, A.; Swindell, R.; Yap, BK.; Sykes, AJ.; Slevin, NJ.; Homer, JJ. et al. (Mar 2013). "Nasopharyngeal carcinoma: a retrospective review of demographics, treatment and patient outcome in a single centre.". Clin Oncol (R Coll Radiol) 25 (3): 171-7. doi:10.1016/j.clon.2012.10.006. PMID 23337060.
  3. Abdel Khalek Abdel Razek, A.; King, A. (Jan 2012). "MRI and CT of nasopharyngeal carcinoma.". AJR Am J Roentgenol 198 (1): 11-8. doi:10.2214/AJR.11.6954. PMID 22194474.
  4. Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 145. ISBN 978-1416002741.
  5. Wenig, BM. (Dec 1999). "Nasopharyngeal carcinoma.". Ann Diagn Pathol 3 (6): 374-85. doi:10.1053/ADPA00300374. PMID 10594290.
  6. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 39. ISBN 978-0781765275.
  7. Gulley ML, Nicholls JM, Schneider BG, Amin MB, Ro JY, Geradts J (April 1998). "Nasopharyngeal carcinomas frequently lack the p16/MTS1 tumor suppressor protein but consistently express the retinoblastoma gene product". Am. J. Pathol. 152 (4): 865–9. PMC 1858242. PMID 9546345. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1858242/.
  8. 8.0 8.1 Plaza, G.; Manzanal, AI.; Fogué, L.; Santón, A.; Martínez-Montero, JC.; Bellas, C. (Mar 2002). "Association of Epstein-Barr virus and nasopharyngeal carcinoma in Caucasian patients.". Ann Otol Rhinol Laryngol 111 (3 Pt 1): 210-6. doi:10.1177/000348940211100304. PMID 11913680.
  9. Singhi, AD.; Califano, J.; Westra, WH. (Feb 2012). "High-risk human papillomavirus in nasopharyngeal carcinoma.". Head Neck 34 (2): 213-8. doi:10.1002/hed.21714. PMID 21484924.