[Rare tumors of the head and neck; on behalf of the REFCOR, the French Network of rare head and neck tumors]

Bull Cancer. 2014 May 1;101(5):411-23. doi: 10.1684/bdc.2014.1956.
[Article in French]


Malignant tumors of the upper aerodigestive tract may be rare by their histology (sarcoma, variants of conventional squamous cell carcinomas) and/or location (sinuses, salivary glands, ear, of various histologies themselves). They represent less than 10% of head and neck neoplasms. The confirmation of their diagnosis often requires a medical expertise and sometimes biomolecular techniques complementary to classical histology and immunohistochemistry. Due to their location, their treatment often requires a specific surgical technique. Radiation therapy is indicated based on histoclinical characteristics common to other head and neck neoplasms but also incorporate grade. Further, the technique must often be adapted to take into account the proximity of organs at risk. For most histologies, chemotherapy is relatively inefficient but current molecular advances may allow to consider pharmaceutical developments in the coming years. The REFCOR, the French Network of head and neck cancers aims to organize and promote the optimal management of these rare and heterogeneous diseases, to promote research and clinical trials.

Keywords: diagnostic; expert; head and neck; management; network; rare tumor.

Publication types

  • English Abstract

MeSH terms

  • Carcinoma, Squamous Cell* / pathology
  • Carcinoma, Squamous Cell* / therapy
  • Ear Neoplasms / pathology
  • Ear Neoplasms / therapy
  • France
  • Humans
  • Otorhinolaryngologic Neoplasms* / pathology
  • Otorhinolaryngologic Neoplasms* / therapy
  • Paranasal Sinus Neoplasms / pathology
  • Paranasal Sinus Neoplasms / therapy
  • Rare Diseases* / pathology
  • Rare Diseases* / therapy
  • Regional Medical Programs / organization & administration
  • Salivary Gland Neoplasms* / pathology
  • Salivary Gland Neoplasms* / therapy
  • Sarcoma* / pathology
  • Sarcoma* / therapy