Current and Emerging Therapies for Advanced Adrenocortical Carcinoma

Oncologist - Tập 16 Số 1 - Trang 36-48 - 2011
Lyndal Tacon1,2, Ruth Prichard3, Patsy S. Soon1,4, Bruce G. Robinson1,2, Roderick Clifton‐Bligh1,2, Stan B. Sidhu1,3
1aCancer Genetics Unit, Hormones & Cancer Group, Kolling Institute of Medical Research, University of Sydney, Sydney, Australia
2bDepartment of Endocrinology, Royal North Shore Hospital, St. Leonards, Australia
3cDepartment of Endocrine and Oncology Surgery, Royal North Shore Hospital, St. Leonards, Australia
4dDepartment of Surgery, Bankstown Hospital and South West Clinical School, University of New South Wales, Sydney, Australia

Tóm tắt

AbstractLearning ObjectivesAfter completing this course, the reader will be able to: Review the role and describe the limitations of conventional therapies for adrenocortical carcinoma.Evaluate the current preclinical molecular research contributing to the rational selection of targeted therapies for adrenocortical carcinoma.CME This article is available for continuing medical education credit at CME.TheOncologist.comAdrenocortical carcinoma (ACC) is a rare but aggressive malignancy with a poor prognosis. Complete surgical resection offers the only potential for cure; however, even after apparently successful excision, local or metastatic recurrence is frequent. Treatment options for advanced ACC are severely limited. Mitotane is the only recognized adrenolytic therapy available; however, response rates are modest and unpredictable whereas systemic toxicities are significant. Reported responses to conventional cytotoxic chemotherapy have also been disappointing, and the rarity of ACC had hampered the ability to undertake randomized clinical studies until the establishment of the First International Randomized Trial in Locally Advanced and Metastatic Adrenocortical Carcinoma. This yet-to-be reported study seeks to identify the most effective first- and second-line cytotoxic regimens. The past decade has also seen increasing research into the molecular pathogenesis of ACCs, with particular interest in the insulin-like growth factor signaling pathway. The widespread development of small molecule tyrosine kinase inhibitors in broader oncological practice is now allowing for the rational selection of targeted therapies to study in ACC. In this review, we discuss the currently available therapeutic options for patients with advanced ACC and detail the molecular rationale behind, and clinical evidence for, novel and emerging therapies.

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