Nasopharyngeal carcinoma

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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, sinonasal undifferentiated carcinoma (SNUC)
IHC p16 -ve, LMP-1 -ve/+ve
Molecular EBER +ve
Site nasopharynx - see head and neck

Signs neck mass
Prevalence uncommon in West

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

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.