Impact of the French ENDOCAN-COMETE Network on Overall Survival in Rare Adrenocortical Carcinomas

Adrenocortical carcinoma (ACC) is a rare cancer. The French ENDOCAN-COMETE network was established in 2009 to coordinate care locally and nationally, and to promote research and education on malignant adrenal tumors. Evidence demonstrating the benefits of this organization is currently lacking.

Patients diagnosed with ACC between 2010 and 2017 were identified from the French Network of Cancer Registries (FRANCIM). Patients were categorized based on referral to the ENDOCAN-COMETE network as either referred at diagnosis (R-ACC) or not referred, or referred late (nR-ACC). Median overall survival (OS) and OS rates at 1, 5, and 10 years were compared between the R-ACC and nR-ACC groups after adjustment for prognostic parameters.

A total of 134 patients with ACC were identified from the FRANCIM registries, corresponding to an incidence of 1.4 cases per million person-years. Ten patients were excluded because of insufficient information regarding their health care pathway. The final analysis included 124 patients (mean age, 53.6 years; female-to-male ratio, 2:1). At diagnosis, 45.2% had European Network for the Study of Adrenal Tumours (ENSAT) stage I-II disease, 48.4% had stage III-IV disease, and 6.4% had an unknown stage; endocrine and/or tumour-related symptoms were present in 62.9% of patients. Among the analyzed patients, 87 (70%) were R-ACC, whereas 37 (30%) were nR-ACC. In patients with localized stage I-II ACC, OS rates were significantly higher in the R-ACC group than in the nR-ACC group: 1-year OS was 92% versus 85%, 5-year OS was 81% versus 55%, and 10-year OS was 75% versus 35%, respectively. This survival advantage remained significant after adjustment for prognostic factors (hazard ratio, 3.9; P=.026). In contrast, among patients with advanced ACC, OS rates were similar between the 2 groups.

Our study demonstrates an OS benefit for patients with stage I-II ACC who were referred early to specialized centers within the ENDOCAN-COMETE network. The combined use of the French cancer registries and dedicated national networks provides the strongest evidence to date demonstrating the impact of such a network.