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Endocrine Abstracts (2013) 32 S17.2 | DOI: 10.1530/endoabs.32.S17.2

1Referral Center for rare adrenal diseases, Cochin Hospital, Paris, France; 2INSERM U1016, Paris, France.

Adrenocortical cancer (ACC) is a rare tumor with an overall poor prognosis. However outcome is heterogeneous and the indication and type of medical therapy should be personalized on the basis of tumor stage and prognostication. The initial diagnostic and extension work-up of an ACC is an important step. Most patients with ENSAT stage 1–3 ACC and some stage 4 ACC will be operated and pathological analysis is important for management. The goals of medical treatment are to control steroid excess and tumor development. Symptomatic treatment and steroidogenesis inhibitors can be used to control the consequences of secreting ACC. Mitotane is usually the drug of choice for both its anticortisolic and adrenolytic effects. However, due to its long delay of action it can be associated in severe cortisol secreting ACC with other drugs like metyrapone and/or ketoconazole, which have a faster effect. Mitotane can be also discussed as adjuvant therapy after complete removal of a tumor for its adrenolytic effect to prevent tumor recurrence. Retrospectives studies have shown a longer disease free survival with adjuvant mitotane, but this has not be a constant finding. One should keep in mind that the probability of recurrence varies among stage 1 and 2 ACC and that development of pathological or molecular markers for risk assessment will help. In case of tumor recurrence or in metastasized ACC, medical therapy with mitotane is used and adapted to its plasma levels. Cytotoxic chemotherapy will be discussed depending on the rate of tumor progression and the response to mitotane. The FIRM-ACT trial has shown, in patients treated with mitotane, a better response rate and progression free survival using etoposide-doxorubicine-cisplatine than streptozotocine. Others cytotoxic chemotherapies and targeted therapies have been used in progressive ACC with lower response rate and will be summarized. Since most ACC overexpress IGF2 the IGFs inhibitors have been developed. International efforts in prospective therapeutic trials are developed in this field. For this goal patient management in coordination with expert centers and networks of clinical research (in Europe are important.

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