Adrenocortical Carcinoma, 40-Year Experience of a Canadian Tertiary Cancer Centre

Abstract

Purpose: To review a single Tertiary Cancer Centre experience in management of ACC treated over a period of 40 years. To review literature foravailable guidelines for treatment of ACC and available evidence for adjuvant RT. Methods: A retrospective chart review for ACC patient treated in single cancer centre between January 1974 and December 2013 was conducted. Patients demographic and tumour characteristics were extracted. Date of referral to Cancer Center and primary and adjuvant treatment modalities were recorded. Outcome data including dates and sites of failure, vital status, and cause of death were collected. Overall survival (OS) and disease free survival (DFS) curves were created using Kaplan Meier test. A Medline search using pub-med, Ovid, and Embase was carried out to review literature regarding the role of RT and any available management guidelines. Results: Out of 81 patients identified in the database, 39 had confirmed ACC. Thirty-two patients had surgical resections including two with M1 disease. Among the patients who did not have surgical resection, 4 patients were metastatic and two were non-resectable or medically inoperable. Out of the 32 patients who had surgeries, 16 had adjuvant systemic treatment including mitotane or concurrent chemo-radiation Only 6 patients had adjuvant RT, of whom 3 are still alive (2 of them alive with distant failure). At median follow up period of 3.8 years, 28 patients died (71%), 10 are alive (25%) and one lost follow up. Of the 22 patients with failure data, 2 had local failure and the rest had distant failure. Conclusion: The current data is insufficient to make treatment recommendation. Collaborative database and consensus on diagnostic and therapeutic guideline is warranted for better identification of optimum management. Adjuvant radiation therapy could be a reasonable option for R1 disease, and randomized controlled trial is needed.