title: Adrenocortical Carcinoma: A Clinician's Perspective.
abstract: Within the category of orphan diseases and rare malignancies, adrenocortical carcinoma (ACC) represents an aggressive entity with high mortality and morbidity. While localized tumors which are diagnosed early can be cured with surgical intervention, there are prognostic factors which predict for micrometastases and consequent recurrent and advanced disease. In such cases, mitotane and cytotoxic chemotherapy have been utilized with a modest degree of benefit. The poor prognosis of recurrent and advanced ACC has underscored the interest in nuanced characterization of ACC cases to guide the personalized use of immunotherapeutic and novel targeted therapies.
authors.affiliation: Dana-Farber Cancer Institute/Brigham and Women's Hospital, Lank Center for Genitourinary Oncology, 450 Brookline Avenue, Boston, MA 02215, USA. Electronic address: Aymen_Elfiky@dfci.harvard.edu.
authors.forename: Aymen
authors.initials: A
authors.lastname: Elfiky
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D018268:Adrenocortical Carcinoma / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003937:Diagnosis, Differential; D006801:Humans; D009386:Neoplastic Syndromes, Hereditary / Q000175:diagnosis
keywords: Adrenal cortex tumors; Adrenocortical carcinoma; Mitotane; Platinum-based chemotherapy; Rare malignancies