Nasopharynx

Overview

Nasopharyngeal Carcinoma

  • Nasopharyngeal carcinoma (NPC) is an uncommon malignancy that is more frequently seen in the Inuit population as well as the ethnic Chinese population particularly from the Guangdong province.1
  • This increased risk is thought to be due to a combination of genetic and environmental factors.
  • Environmental factors that are associated with NPC include salted fish (thought to be related to nitrosamines) and Epstein-Barr virus (EBV) infection.

Classification of NPC

  • Pathologically, the most recent World Health Organization (WHO) Classification of Tumours (4th Edition, 2017) has divided NPC into the following groups:2
    1. Keratinizing squamous cell carcinoma
    2. Non-keratinizing carcinoma
      • Differentiated
      • Undifferentiated
    3. Basaloid squamous cell carcinoma

Changes from the AJCC 7th Edition

T-Categorization

  • T0 has been added to the classification system for situations where there is EBV positive metastatic carcinoma of unknown primary.
  • References to the masticator space and infratemporal fossa have been removed and replaced with consistent definitions that refer to specific soft tissue and bony structures.

N-Categorization

  • Retropharyngeal nodal involvement (unilateral or bilateral) is now clearly N1.
  • Subcategories within N3 (N3a, N3b) have now been eliminated.
  • Involvement supraclavicular fossa have been removed as a criterion for N3 disease. Instead, the inferior border of the cricoid cartilage is used as a demarcating point.

Group Staging

  • Stage IVA and IVB have been merged into IVA.
  • Stage IVC (the presence of distant metastasis) has been downstaged to IVB.

T-Categorization
T Category Criteria
TX

Primary tumour cannot be assessed

T0

No primary tumour identified, but EBV positive cervical nodal involvement.

Tis

Carcinoma in situ.

T1
  • Tumour involving the nasopharynx +/- nasal cavity +/- oropharynx without involvement of the parapharyngeal space
T2
  • Extension to the parapharyngeal space.
  • OR involvement of surrounding soft tissue structures (medial pterygoid muscle, lateral pterygoid muscle, prevertebral muscles).
T3
  • Tumour involvement of bony structures (medial or lateral pterygoid plates, cervical vertebrae, skull base, paranasal sinuses).
T4
  • Tumor with intracranial extension, involvement of cranial nerves, hypopharynx, orbit, parotid gland, and/ or extensive soft tissue infiltration beyond the lateral surface of the lateral pterygoid muscle

General Rules

  • Mucosal involvement only of the nasopharynx, nasal cavity, or oropharynx by NPC is considered T1.
  • Involvement of the parapharyngeal space or soft tissue structures (medial/lateral pterygoid muscle, prevertebral muscle) up-categorizes to T2
  • Involvement of bony structures (medial/lateral pterygoid plates, cervical vertebrae, skull base, paranasal sinuses) up-categorizes to T3
  • Extension beyond these structures (skull base, orbit, hypopharynx, etc) up-categorizes to T4
  • Previously the AJCC 7th edition guidelines referenced the infratemporal fossa (which has mainly bony limits) and the masticator space (which has fascial limits) in their staging. This had been confusing as the two areas are similar but not identical.

N-Categorization
N Category Criteria
NX

Regional lymph nodes cannot be assessed.

N0

No regional lymph node metastasis.

N1
  • Unilateral or retropharyngeal nodal involvement that is ≤ 6 cm in maximal dimension and above the caudal border of the cricoid cartilage.
N2
  • Bilateral nodal involvement that is ≤ 6 cm in maximal dimension and above the caudal border of the cricoid cartilage.
N3
  • Nodal involvement that is >6 cm in maximal dimension.
  • OR nodal involvement that extends below the caudal border of the cricoid cartilage.

General Rules

  • Unilateral or retropharyngeal nodal involvement up-categorizes to N1
  • Bilateral nodal involvement up-categorizes to N2
  • If there is any nodal involvement > 6 cm or nodes that extend below the caudal border of the cricoid then this is categorized as N3.

M-Categorization
M Category Criteria
M0

No distant metastasis

M1

Distant metastasis present


Group Staging
N0
M0
N1
M0
N2
M0
N3
M0
Any N
M1
Tis 0
T0 II III IVA IVB
T1 I II III IVA IVB
T2 II II III IVA IVB
T3 III III III IVA IVB
T4 IVA IVA IVA IVA IVB
  1. Bailey, B. J., Johnson, J. T., & Rosen, C. A. (2014). Chapter 120 – Nasopharyngeal Carcinoma. In Bailey's Otolaryngology – Head & Neck Surgery. Wolters Kluwer, Lippincott Williams et Wilkins.  

  2. Grandis, El-Naggar AK Chan JK, and JR Takata T. Slootweg PJ WHO. "Classification of Head and Neck Tumours." (2017).