Advanced Adrenocortical Carcinoma: Current Perspectives on Medical Treatment.

摘要: Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy. For stage I II tumors, surgery curative option, but even in these cases recurrence frequent. Practical guidelines advocate combination of mitotane with etoposide, doxorubicin, cisplatin as first-line therapy for metastatic adrenocortical carcinoma. However, this scheme presents limited efficacy high toxicity. The use Immune Checkpoint Inhibitors (ICI) multi-Tyrosine Kinase (mTKI) has modified the approach multiple malignancies. expectation their applicability on advanced role new therapies persists unclear. This article provides short summary last years' findings targeting outcomes, limitations, adverse effects therapeutic approaches. results recent trials case series pointed pembrolizumab most promising drug among therapies. It often used ICI one presenting best less related when comparison to standard treatment mitotane. Hereafter, identification specific molecular biomarkers or immune profiles associated mTKI good response will facilitate selection candidates So far, microsatellite instability Lynch Syndrome germline mutations are suggested predictive response. Contrarywise, cortisol secretion been more ACC tumors potentially poor responses immunotherapy.