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J Clin Endocrinol Metab. 2014 Feb;99(2):455-61. doi: 10.1210/jc.2013-2856. Epub 2013 Dec 3.

Adjuvant therapies and patient and tumor characteristics associated with survival of adult patients with adrenocortical carcinoma.

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Department of Internal Medicine (T.E., G.D.H.), Division of Metabolism, Endocrinology, and Diabetes, Department of Radiation Oncology (A.S., S.J.), and Division of Endocrine Surgery (B.S.M.), Section of General Surgery, University of Michigan Hospital and Health Systems, Ann Arbor, Michigan 48106; and University of Michigan Medical School (A.R.W.), Ann Arbor, Michigan 48109.



Adrenocortical carcinoma is a rare malignant endocrine neoplasia. Studies regarding outcome and prognostic factors rely on fairly small studies. Here we summarize the experience with patients with a diagnosis of adrenocortical carcinoma from a large tertiary referral center.


The objective of the study was to identify prognostic factors in patients with adrenocortical carcinoma and evaluate adjuvant treatment strategies.


Patient data were collected in a retrospective single-center study. Epidemiological, patient, and tumor characteristics were analyzed for prognostic factors regarding overall and recurrence-free survival in Cox regression models (multivariable and univariable).


Three hundred ninety-one adult patients with the diagnosis of adrenocortical carcinoma were identified. Median overall survival was 35.2 months. Cortisol production [hazard ratio (HR) 1.4, HR 1.5], tumor stage (HR stage 3 of 2.1 and 2.1, HR stage 4 of 4.8), and tumor grade (HR 2.4 and 2.0) were identified as negative prognostic factors (HR for death, HR for recurrence). Mitotane therapy increases recurrence-free survival, an effect that was significantly further improved by adjuvant radiation therapy but did not impact overall survival. Patients with open adrenalectomy had improved overall survival.


This study increases the evidence for adverse risk factors (cortisol production, high tumor stage, and high tumor grade) and suggests the following therapy approach: adrenocortical carcinoma patients should be treated with open adrenalectomy. Adjuvant therapy, particularly mitotane therapy in conjunction with radiation, should be considered to delay tumor recurrence.

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