Context: Adrenocortical carcinoma is a rare malignant endocrine neoplasia. Studies regarding outcomeandprognostic factors relyonfairly small studies. Herewesummarize the experience with patients with a diagnosis of adrenocortical carcinoma from a large tertiary referral center. Objective: The objective of the study was to identify prognostic factors in patients with adrenocortical carcinoma and evaluate adjuvant treatment strategies. Design: Patient datawerecollected in a retrospective single-center study. Epidemiological, patient, and tumor characteristics were analyzed for prognostic factors regarding overall and recurrencefree survival in Cox regression models (multivariable and univariable). Results: 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. Conclusions: 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. Copyright © 2014 by the Endocrine Society.
CITATION STYLE
Else, T., Williams, A. R., Sabolch, A., Jolly, S., Miller, B. S., & Hammer, G. D. (2014). Adjuvant therapies and patient and tumor characteristics associated with survival of adult patients with adrenocortical carcinoma. Journal of Clinical Endocrinology and Metabolism, 99(2), 455–461. https://doi.org/10.1210/jc.2013-2856
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