title: New insights and future perspectives in the therapeutic strategy of adrenocortical carcinoma (Review).
abstract: 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.
authors.affiliation: Endocrinology, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Faculty of Medicine and Psychology, Sapienza University of Rome, 00189 Rome, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Stigliano
authors.affiliation: Endocrinology, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Faculty of Medicine and Psychology, Sapienza University of Rome, 00189 Rome, Italy.
authors.forename: Lidia
authors.initials: L
authors.lastname: Cerquetti
authors.affiliation: Endocrinology, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Faculty of Medicine and Psychology, Sapienza University of Rome, 00189 Rome, Italy.
authors.forename: Pina
authors.initials: P
authors.lastname: Lardo
authors.affiliation: CNR, Institute of Molecular Biology and Pathology, Sapienza University of Rome, 00161 Rome, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Petrangeli
authors.affiliation: Endocrinology, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, Faculty of Medicine and Psychology, Sapienza University of Rome, 00189 Rome, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Toscano
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000188:drug therapy*; D018268:Adrenocortical Carcinoma / Q000188:drug therapy*; D000818:Animals; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D006801:Humans
chemical_list: D000970:Antineoplastic Agents