Head and neck adenoid cystic carcinoma: A prospective multicenter REFCOR study of 95 cases

Eur Ann Otorhinolaryngol Head Neck Dis. 2016 Feb;133(1):13-7. doi: 10.1016/j.anorl.2015.09.009. Epub 2015 Oct 20.


Objectives: To describe the clinical, histological and therapeutic characteristics of a prospective multicenter series of 95 head and neck adenoid cystic carcinoma patients, and to determine any prognostic factors for disease-free survival.

Patients and methods: Ninety-five patients with adenoid cystic carcinoma were included in the Réseau d'Expertise Français Des Cancers ORL Rares (REFCOR, French Rare Head and Neck Cancer Expert Network) database between 2009 and 2012. The primary site was the salivary glands in 39 cases, sinus cavities (including hard palate) in 36 cases, pharynx-larynx-trachea in 14 cases, and lips and oral cavity in 4 cases. The tumor was stage I in 15% of cases, stage II in 23%, stage III in 26% and stage IV in 36%. Nine patients had cervical lymph node involvement and 5 had metastases at diagnosis. Fifty-six percent of patients were managed by surgery with postoperative radiation therapy. During follow-up, 3 patients died, 9 developed metastases and 12 showed recurrence or local progression.

Results: Mean follow-up was 18 months. On univariate analysis, disease-free survival correlated with T stage (P=0.05), N stage (P=0.003), resection margins (P=0.04), lymph node involvement on histology (P=0.01), and absence of chemotherapy (P=0.03). On multivariate analysis, disease-free survival correlated with T stage (P=0.01), N stage (P=0.09) and surgery (P=0.005).

Conclusion: The essential issue in adenoid cystic carcinoma is long-term control. The present results confirm that the reference attitude is radical surgical resection for optimal local control. Adjuvant radiation therapy did not emerge as a prognostic factor. This study also provides a starting-point for translational studies in pathology and genetics.

Keywords: Adenoid cystic carcinoma; Prognostic factors; Salivary glands.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Carcinoma, Adenoid Cystic / mortality*
  • Carcinoma, Adenoid Cystic / pathology
  • Carcinoma, Adenoid Cystic / therapy*
  • Disease-Free Survival
  • Female
  • France / epidemiology
  • Head and Neck Neoplasms / mortality*
  • Head and Neck Neoplasms / pathology
  • Head and Neck Neoplasms / therapy*
  • Humans
  • Lymphatic Metastasis
  • Male
  • Middle Aged
  • Prognosis
  • Prospective Studies
  • Radiotherapy, Adjuvant
  • Young Adult