Surgery for adrenocortical carcinoma in The Netherlands: analysis of the national cancer registry data

Objective: Adrenocortical carcinoma (ACC) is a rare disease with an estimated incidence of one to two cases per 1 million inhabitants. The Dutch Adrenal Network (DAN) was initiated with the aim to improve patient care and to stimulate scientific research on ACC. Currently, not all patients with ACC are treated in specialized DAN hospitals. The objective of the current investigation was to determine whether there are differences in survival between patients operated on in DAN hospitals and those operated on in non-DAN hospitals. Design: The study was set up as a retrospective and population-based survival analysis. Methods: Data on all adult ACC patients diagnosed between 1999 and 2009 were obtained from The Netherlands Cancer Registry (NCR). Overall survival was calculated and a comparison was made between DAN and non-DAN hospitals. Results: The NCR contained data of 189 patients. The median survival of patients with European Network for the Study of Adrenal Tumors stages I-III disease was significantly longer for patients operated on in a DAN hospital (n=46) than for those operated on in a non-DAN hospital (n=37, 5-year survival 63 vs 42%). Survival remained significantly different after correction for sex, age, year of diagnosis, and stage of disease in the multivariate analysis (hazard ratio 1.96 (95% CI 1.01-3.81), P=0.047). Conclusion: The results associate surgery in a DAN center with a survival benefit for patients with local or locally advanced ACC. We hypothesize that a multidisciplinary approach for these patients explains the observed survival benefit. These findings should be carefully considered in view of the aim for further centralization of ACC treatment.