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Treatment of adrenocortical carcinoma

Authors
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

INTRODUCTION

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".)

STAGING

The best staging system for adrenocortical carcinoma (ACC) is evolving. Although there is a tumor node metastasis (TNM) staging system available from the American Joint Commission on Cancer (AJCC)/International Union Against Cancer (UICC) (table 1), most contemporary treatment studies use the modification for stage groupings 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 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].

PRIMARY TREATMENT

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 (according to the European Network for the Study of Adrenal Tumors [ENSAT] stage groupings (table 2)) who are surgical candidates, we recommend complete surgical resection as initial therapy.

                                      

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Literature review current through: Nov 2016. | This topic last updated: Tue Oct 11 00:00:00 GMT+00:00 2016.
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