Adrenocortical Carcinoma: Patterns of Care and Role of Adjuvant Radiation Therapy—A Population-Based Study and Review of the Literature

PURPOSE: We reviewed the experience of a tertiary cancer centre in the management of adrenocortical carcinoma (acc) treated over 40 years. We also searched the literature for guidelines related to the treatment of acc and for evidence for adjuvant radiation therapy (rt). METHODS: In a retrospective chart review, acc patients treated between January 1974 and December 2013 were identified, and patient demographics and tumour characteristics were extracted. Outcomes data, including dates and sites of failure, vital status, and cause of death, were collected. Overall survival was estimated using the Kaplan- Meier method. A medline search using PubMed, Ovid, and embase was used to review the literature about the role of rt and any available management guidelines for acc. RESULTS: Of 81 patients identified during the chart review, 39 had confirmed acc. In 32 patients, surgical resection was performed, including in 2 patients with M1 disease. Of those 32 patients, 16 received adjuvant systemic treatment (mitotane or concurrent chemoradiation). Only 6 patients received adjuvant rt, of whom 3 are still alive (2 living with distant failure). At a median follow-up of 3.8 years, 28 patients had died (72%), 10 were living (26%), and 1 had been lost to follow-up. Of the 22 patients for whom failure data were available, 2 experienced local failure, and the rest, distant failure. CONCLUSIONS: The current data are insufficient to make treatment recommendations. Use of collaborative databases and consensus about diagnostic and therapeutic guidelines are warranted for better identification of optimum management. Adjuvant rt could be a reasonable option for R1 disease, but further research is needed.