Malignant Tumors of the Adrenal Gland

Abstract

Overview Optimal evaluation and treatment of malignant or potentially malignant adrenal tumors including pheochromocytoma/paraganglioma and adrenal cortical carcinoma can be complex. It requires appropriate diagnostic imaging and endocrine hormone evaluation, and benefits from expert multidisciplinary coordination, by medical oncologists, endocrinologists, and surgeons, as well as additional specialists, for example genetic counselors. Recent advances in diagnostic imaging, including functional imaging such as PET, have facilitated early detection and diagnosis, enhanced the accuracy of staging, and improved treatment planning. Good‐risk patients with resectable localized, recurrent, or limited metastatic disease are usually recommended to undergo either minimally invasive or open surgery. Advances in minimally invasive and open surgical techniques have improved surgical outcomes. There is no defined role for adjuvant therapy following resection of pheochromocytoma/paraganglioma. Adjuvant therapy for patients with adrenal cortical carcinoma, most commonly single‐agent mitotane, is considered selectively. Systemic treatment options for patients with locally recurrent, unresectable, or metastatic malignant adrenal carcinoma include targeted treatments for patients with metastatic pheochromocytoma/paraganglioma, and combination chemotherapy with or without mitotane for patients with adrenal cortical carcinoma. Radiation therapy for patients with malignant adrenal tumors of either histology is also employed selectively. Emerging genetic and molecular data, as well as clinical trials, increasingly inform treatment strategies and choice of systemic therapy.