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J Clin Endocrinol Metab. 2019 Jul 30. pii: jc.2019-00915. doi: 10.1210/jc.2019-00915. [Epub ahead of print]

National Treatment Practice for Adrenocortical Carcinoma: Have they changed and have we made any progress?

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Division of Surgical Oncology, Department of Surgery, Rush University Medical Center, Chicago, IL.
Division of Transplant, Department of Surgery, Rush University Medical Center, Chicago, IL.
Cantonal Hospital Winterthur, Department of Surgery, Winterthur, Zurich, Switzerland.
University of Zurich, Institute of Physiology, Zurich, Switzerland.
Division of General Surgery, Department of Surgery, Stanford University, Stanford, CA.
Endocrine Surgery Research Program, Division of General Surgery and Surgical Oncology, Department of Surgery, the University of Chicago, Chicago, IL.



The NCDB was used to examine treatment patterns and survival in patients with adrenocortical carcinoma from 2006-2015. Adjuvant chemotherapy has been used more frequent for patients with resectable ACC since 2007. Survival for patients who undergo surgery with adjuvant chemotherapy has improved but remains low.Accepted for podium presentation at the 2018 Clinical Congress of the American College of Surgeons, Boston, Massachusetts, October 2018.


Adrenocortical carcinoma (ACC) is a rare malignancy with a dismal prognosis. Two landmark trials, published in 2007 and 2012, showed efficacy for adjuvant mitotane in resectable ACC and Etoposide/Doxorubicin/Cisplatin plus mitotane for unresectable ACC, respectively. In this study, we used the National Cancer Database to examine whether treatment patterns and outcomes changed after these trials.


Patients diagnosed with ACC from 2006-2015 were identified. Treatment modalities were compared within that group, and with a historical cohort (1985-2005). Chi-square tests were performed and Cox proportional hazards models were created.


2752 patients from 2006-2015 were included. 38% of patients (1042) underwent surgery alone and 31% (859) underwent surgery with adjuvant therapy. Overall five-year survival for all stages after resection was 43% (median 41 months) in the contemporary cohort and 39% (median 32 months) in the historical cohort. After 2007, patients who underwent surgery were more likely to receive adjuvant chemotherapy (p = 0.005), and five-year survival with adjuvant chemotherapy improved (41% vs 25%, p = 0.02). Survival did not improve, however, in patients with unresectable tumors after 2011, compared to 2006-2011 (p = 0.79). Older age, tumor size ≥ 10cm, distant metastases, and positive margins were associated with lower survival after resection (HR range: 1.39-3.09, p < 0.03).


Adjuvant therapy has been used more frequently in patients with resected ACC since 2007, and survival for those patients has improved, but remains low. More effective systemic therapies for patients with ACC and especially those with advanced stages are desperately needed.


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