Update in Adrenocortical Carcinoma

摘要: Adrenocortical carcinoma (ACC) is an orphan malignancy that has attracted increasing attention during the last decade. Here we provide update on advances in field since our review published this journal 2006. The Wnt/β-catenin pathway and IGF-2 signaling have been confirmed as frequently altered pathways ACC, but recent data suggest they are probably not sufficient for malignant transformation. Thus, major players pathogenesis still unknown. For diagnostic workup, comprehensive hormonal assessment detailed imaging required because most ACCs, evidence autonomous steroid secretion can be found computed tomography or magnetic resonance (if necessary, combined with functional imaging) differentiate benign from adrenocortical tumors. Surgery potentially curative localized recommend a complete resection including lymphadenectomy by expert surgeon. pathology report should demonstrate origin of lesion (eg, steroidogenic factor 1 staining) Weiss score, status, quantitation proliferation marker Ki67 to guide further treatment. Even after surgery, recurrence frequent adjuvant mitotane treatment improves outcome, uncertainty exists whether all patients benefit therapy. In advanced standard care. Based FIRM-ACT trial, plus etoposide, doxorubicin, cisplatin now established first-line cytotoxic However, will experience progress require salvage therapies. new concepts urgently needed. ongoing international efforts "-omic approaches" next-generation sequencing improve understanding hopefully lead better