Adrenocortical cancer is a rare and heterogenous malignancy with incompletely understood pathogenesis and poor prognosis. Sometimes patients present with hormonal excess symptoms (e.g. virilization, Cushings syndrome) or local symptoms consistent with abdominal space-occupation (median tumor size at the time of diagnosis may be >10 cm). Three cases are presented to give an overview of how adrenocortical cancer is currently managed. Tumors typically appear inhomogenous on computed tomography or magnetic resonance imaging showing signs of necrosis, irregular borders and they differ from benign adenomas by their low fat content. Hormonal evaluations are important additives for the diagnosis. All patients with suspected adrenocortical carcinoma should be carefully evaluated for signs and symptoms of hormonal syndromes Prognosis depends on many factors. Complete surgical resection is the treatment of choice. Mitotane is given when surgery is not possible, after incomplete resection or in cases of metastatic disease. Chemotherapeutic combination is frequently used (etoposid, doxorubicin and cisplatin plus mitotane or streptozotocin plus mitotane). New treatments, such as insulin-like growth factor I receptor antibodies, tyrosine-kinase inhibitors and antiangiogenic compounds, are now being intensively investigated to find more effective therapies for this extremly severe malignant neoplasia.
24 - 28 Apr 2010
European Society of Endocrinology