Twelve years after: The french national network on rare head and neck tumours (REFCOR)

Oral Oncol. 2024 Apr:151:106762. doi: 10.1016/j.oraloncology.2024.106762. Epub 2024 Mar 20.

Abstract

Background: Rare cancers constitute less than 10% of head and neck cancers and lack sufficient evidence for standardized care. The French Rare Head and Neck Cancer Expert Network (REFCOR) as established a national database to collect data on these rare cancers. This study aims to describe patient and tumour characteristics in this database.

Methods: Prospective data collection was conducted across multiple centers. Survival analyses were performed using Kaplan Meier method and Log Rank test. Odds ratios were used for comparing proportions.

Results: A total of 7208 patients were included over a period of 10 years. The most frequent histologies were: Not Otherwise Specified (NOS) adenocarcinoma 13 %, adenoid cystic carcinoma 12 %, squamous cell carcinoma of rare locations 10 %, mucoepidermoid carcinoma 9 %, intestinal-type adenocarcinoma (8 %). Tumours were located in sinonasal area (38 %); salivary glands (32 %); oral cavity / oropharynx / nasopharynx (16 %); larynx / hypopharynx (3 %); ears (1 %); others (3 %). Tumours were predominantly classified as T4 (23 %), N0 (54 %), and M0 (62 %). Primary treatment approach involved tumour resection (78 %) and / or radiotherapy (63 %). Patients with salivary gland cancers exhibited better 5-year overall survival (OS) rates (p < 0.05), and lower recurrence rates compared to patients with sinonasal, laryngeal/ hypopharyngeal cancers. No significant differences were observed in the other comparisons. Acinar cell carcinoma demonstrated the best OS while mucous melanoma had the poorest prognosis.

Conclusion: Melanoma, carcinoma NOS, and sinonasal undifferenciated carcinoma still have poor prognoses. Efforts are being made, including training and guidelines, to expand network coverage (REFCOR, EURACAN), improve data collection and contribute to personalized therapies.

Keywords: Head and Neck cancers; Rare cancer network; Rare cancers; Salivary gland cancer; Sinonasal cancer.

MeSH terms

  • Adenocarcinoma*
  • Carcinoma, Adenoid Cystic* / pathology
  • Head and Neck Neoplasms* / epidemiology
  • Head and Neck Neoplasms* / therapy
  • Humans
  • Melanoma*
  • Paranasal Sinus Neoplasms* / pathology
  • Salivary Gland Neoplasms* / pathology