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Nasopharyngeal Carcinoma

last modified on: Mon, 03/25/2024 - 08:51

see also: Nasopharynx Rads or Nasopharyngeal Carcinoma Rads

Note: last updated before 2015

Overview

i. Nasopharyngeal carcinoma is a rare presentation of head and neck squamous cell carcinoma that differs from typical squamous cell cancers of the head and neck in etiology, histology and treatment response.

ii. Nasopharyngeal carcinomas account for a 70% majority of malignancies arising in the nasopharynx.

iii. The occult location and invasive nature of these neoplasms often results in incidental discovery and presentation at advanced stages.

iv. Nasopharyngeal carcinomas are categorized by two main histologic sub-types:

Keratinizing: Sporadic form of nasopharyngeal carcinoma, most consistent with typical head and neck squamous neoplasm, and associated with alcohol and tobacco use

Non-keratinizing: Strongly associated with EBV, Eastern Asian descent, and the consumption of certain food items.

v. External beam radiation and chemotherapy are the mainstays in treatment; surgery has a limited role. 

Differential Diagnosis

  • Prominent, but normal, adenoidal tissue      
  • Nasal Polyp
  • Nasopharygeal lymphoma
  • Adenocarcinoma
  • Adenoid cystic carcinoma
  • Melanoma
  • Sarcoma (rhabdomyo-, carcino-)

Epidemiology/Etiology

There are 80,000 incident cases of nasopharyngeal carcinoma worldwide annually. Significantly more cases occur in Southeast Asia, and parts of the Middle East and North Africa, such that the condition is considered endemic to these areas. Emigration out of endemic areas does not reduce the risk, and even children of emigrants carry an increased risk for nasopharyngeal carcinoma in non-endemic areas.

Other significant factors linked to nasopharyngeal carcinoma include:

  • Male sex (3-fold higher risk than women)
  • Increased age
  • Tobacco and alcohol use
  • Regular consumtion of salt-cured foods 
  • Epstein-Barr Virus

Clinical Presentation

Symptoms caused by lesions of the anatomically occult nasopharynx are typically non-specific and frequently overlooked by patients leading to late stage diagnoses. Indeed, multiple reviews of nasopharyngeal carcinoma cases report 55-69% late stage (III or IV) diagnosis, 28-44% of patients had painless neck mass as chief complaint, and average time from onset of symptoms to diagnosis ranged from 1.3 to 3.4 months. Symptoms related to nasopharyngeal carcinoma are often unalarming and underwhelming to patients, contributing to the late presentation.

Common presenting signs and symptoms include:

  • Painless neck mass
  • Nasal congestion
  • Epistaxis
  • Unilateral hearing loss
  • Unilateral ear pressure/fullness
  • Headache

Evaluation

Because of the relatively low incidence and usually non-specific presenting symptoms, physicians should keep nasopharyngeal carcinoma on their differential diagnosis for all of the associated symptoms. The presence of these signs or symptoms or the incidental finding of nasopharyngeal mass along with a history of smoking, the use of alcohol, family history of nasopharyngeal carcinoma, personal history of head and neck cancer, descent from endemic areas, history of EBV illness, or regular consumption of salt-cured foods should raise concern for the potential of nasopharyngeal carinoma and prompt an appropriate evaluation.

  • Cranial nerve exam: Nasopharyngeal carcinomas are aggressive lesions and frequently invade the skull base and cranial nerves. Cranial nerves III, V, and VI are most frequently involved.
  • Flexible endoscopic nasopharyngeal scope: Nasopharyngeal carcinoma most frequently arises in the fossa of Rosenmuller and may occur as small to large masses covered in normal appearing to ulcerated mucosa or may occur as fungating ulcerated masses. Purulent or bloody secretions may be present.

References (none for above)

References to guide future update

Chen YP, Chan ATC, Le QT, Blanchard P, Sun Y, Ma J. Nasopharyngeal carcinoma. Lancet. 2019 Jul 6;394(10192):64-80. doi: 10.1016/S0140-6736(19)30956-0. Epub 2019 Jun 6. PMID: 31178151.

Wong KCW, Hui EP, Lo KW, Lam WKJ, Johnson D, Li L, Tao Q, Chan KCA, To KF, King AD, Ma BBY, Chan ATC. Nasopharyngeal carcinoma: an evolving paradigm. Nat Rev Clin Oncol. 2021 Nov;18(11):679-695. doi: 10.1038/s41571-021-00524-x. Epub 2021 Jun 30. PMID: 34194007.