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Cytotoxic treatment of adrenocortical carcinoma.

Journal article
Authors Håkan Ahlman
Amir Khorram-Manesh
Svante Jansson
Bo Wängberg
Ola Nilsson
C E Jacobsson
Sven Lindstedt
Published in World journal of surgery
Volume 25
Issue 7
Pages 927-33
ISSN 0364-2313
Publication year 2001
Published at Institute of Laboratory Medicine
Institute of Laboratory Medicine, Dept of Pathology
Institute of Surgical Sciences, Department of Surgery
Pages 927-33
Language en
Keywords Adrenal Cortex Neoplasms, drug therapy, Adrenocortical Carcinoma, drug therapy, Antineoplastic Agents, therapeutic use, Cisplatin, therapeutic use, Doxorubicin, therapeutic use, Etoposide, therapeutic use, Humans, Mitotane, therapeutic use
Subject categories Surgery


Adrenocortical carcinoma (ACC) is a rare, aggressive tumor that is often detected in an advanced stage. Medical treatment with the adrenotoxic drug mitotane has been used for decades, but critical prospective trials on its role in residual disease or as an adjuvant agent after surgical resection are still lacking. The concept of a critical threshold plasma level of the drug must be confirmed in controlled studies. Because individual responsiveness cannot be predicted, the use mitotane is still advised for nonresectable disease. In case of cortisol or other steroid overproduction, several drugs (e.g., ketoconazole or aminoglutethimide) may be used. Chemotherapy with single agents (e.g., doxorubicin or cisplatin) have been disappointing, with low response rates (< 30%) and a short response duration. Part of this refractoriness may be explained by the fact that ACC tumors express the multidrug-resistance gene MDR-1. Chemotherapy with multiple agents has been tested in smaller series and has resulted in significant side effects. The best results were achieved by the combination of etoposide, doxorubicin, and cisplatin associated with mitotane, achieving a response rate of 54%, including individual complete responses. To be able to make progress in treating advanced ACC disease, adjuvant multicenter trials must be encouraged. When mitotane-based therapies are used, monitored drug levels are mandatory.

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