PMID- 28184938 OWN - NLM STAT- MEDLINE DCOM- 20170411 LR - 20170411 IS - 1791-2431 (Electronic) IS - 1021-335X (Linking) VI - 37 IP - 3 DP - 2017 Mar TI - New insights and future perspectives in the therapeutic strategy of adrenocortical carcinoma (Review). PG - 1301-1311 LID - 10.3892/or.2017.5427 [doi] AB - Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an incidence ranging from 0.7 to 2.0 cases/million people per year. Hypercortisolism represents the most common clinical presentation in many patients although, less frequently, some ACC secreting androgens and estrogens are even more pathognomonic compared to cortisol secretion. Currently, radical surgery, when feasible, is still the only curative therapy. Mitotane, an adrenolytic drug, is used in the adjuvant setting and in combination with chemotherapy drugs in metastatic disease. The use of radiotherapy remains controversial, being indicated only in selected cases. New targeted therapies, such as insulin growth factor-1 (IGF-1), mammalian-target of rapamycin (m-TOR), vascular endothelial growth factor (VEGF) inhibitors and others, have recently been investigated with disappointing clinical results. The partial effectiveness of current treatments mandates the need for new therapeutic strategies against this tumor. FAU - Stigliano, Antonio AU - Stigliano A AD - Endocrinology, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Faculty of Medicine and Psychology, Sapienza University of Rome, 00189 Rome, Italy. FAU - Cerquetti, Lidia AU - Cerquetti L AD - Endocrinology, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Faculty of Medicine and Psychology, Sapienza University of Rome, 00189 Rome, Italy. FAU - Lardo, Pina AU - Lardo P AD - Endocrinology, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Faculty of Medicine and Psychology, Sapienza University of Rome, 00189 Rome, Italy. FAU - Petrangeli, Elisa AU - Petrangeli E AD - CNR, Institute of Molecular Biology and Pathology, Sapienza University of Rome, 00161 Rome, Italy. FAU - Toscano, Vincenzo AU - Toscano V AD - Endocrinology, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Faculty of Medicine and Psychology, Sapienza University of Rome, 00189 Rome, Italy. LA - eng PT - Journal Article PT - Review DEP - 20170207 PL - Greece TA - Oncol Rep JT - Oncology reports JID - 9422756 RN - 0 (Antineoplastic Agents) SB - IM MH - Adrenal Cortex Neoplasms/*drug therapy MH - Adrenocortical Carcinoma/*drug therapy MH - Animals MH - Antineoplastic Agents/*therapeutic use MH - Humans EDAT- 2017/02/12 06:00 MHDA- 2017/04/12 06:00 CRDT- 2017/02/11 06:00 PHST- 2016/08/03 00:00 [received] PHST- 2016/12/20 00:00 [accepted] PHST- 2017/02/11 06:00 [entrez] PHST- 2017/02/12 06:00 [pubmed] PHST- 2017/04/12 06:00 [medline] AID - 10.3892/or.2017.5427 [doi] PST - ppublish SO - Oncol Rep. 2017 Mar;37(3):1301-1311. doi: 10.3892/or.2017.5427. Epub 2017 Feb 7.