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Treatment of adrenocortical carcinoma

André Lacroix, MD
Gary D Hammer, MD, PhD
Section Editors
Lynnette K Nieman, MD
Alberto S Pappo, MD
Deputy Editors
Kathryn A Martin, MD
Sadhna R Vora, MD


Adrenocortical carcinomas (ACCs) are rare and frequently aggressive tumors that may be functional (hormone-secreting) and cause Cushing's syndrome and/or virilization, or nonfunctional and present as an abdominal mass or as an incidental finding.

The management of ACC will be discussed here. The clinical presentation and diagnostic evaluation of patients with adrenal masses, the staging workup for ACC, and the management of benign adrenal adenomas are reviewed separately. (See "Clinical presentation and evaluation of adrenocortical tumors" and "The adrenal incidentaloma".)


The staging system for adrenocortical carcinoma (ACC) is evolving. The eighth edition of the tumor, node, metastases (TNM) staging system, stage IV has now become harmonized with the one previously proposed by the European Network for the Study of Adrenal Tumors (ENSAT), given its superior prognostic stratification with the restriction of stage IV tumors to those having distant metastases (table 1 and table 2). (See "Clinical presentation and evaluation of adrenocortical tumors", section on 'Staging'.)

A modification has been proposed for the ENSAT staging system that incorporates histologic grade of differentiation (table 3) [1]. In one report, incorporating patient age (>55 years) into available staging systems appeared to better predict overall survival in patients with stage I and II ACC [2].


Initial surgery — Complete surgical resection is the only potentially curative treatment for adrenocortical carcinoma (ACC) [3]. For patients with potentially resectable stage I to III disease who are surgical candidates, we recommend complete surgical resection as initial therapy.


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