Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. ACC is capable of secreting excess adrenocortical hormones, which can compound morbidity and compromise clinical outcomes. By the time most ACCs are diagnosed, there is usually locoregional or metastatic disease. Surgery is the most important treatment to offer possibility of cure or prolong survival. Several adjuvant therapies are used depending on grade and stage of the tumor and other patient-related factors. This review provides an overview of treatment approaches for ACC, highlighting evidence to support each treatment and acknowledging where more data and research are needed to improve care.
Surgical pathology clinics. 2019 Sep 27 [Epub]
Anand Vaidya, Matthew Nehs, Kerry Kilbridge
Division of Endocrinology Diabetes, and Hypertension, Department of Medicine, Center for Adrenal Disorders, Brigham and Women's Hospital, Harvard Medical School, 25 Shattuck Street, Boston, MA 02115, USA. Electronic address: ., Brigham and Women's Hospital, Harvard Medical School, 25 Shattuck Street, Boston, MA 02115, USA; Department of Surgery, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02215, USA., Brigham and Women's Hospital, Harvard Medical School, 25 Shattuck Street, Boston, MA 02115, USA; Department of Surgery, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02215, USA; Lank Center for Genitourinary Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02215, USA.