Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO–EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up
Martin Faßnacht, Guillaume Assié, Eric Baudin, G. Eisenhofer, Christelle de la Fouchardière, Harm R. Haak, Ronald R. de Krijger, F. Porpiglia, Massimo Terzolo, Alfredo Berruti
Abstract
Two different primary malignancies can arise from the adrenal gland: adrenocortical carcinoma (ACC) from the adrenal cortex and malignant phaeochromocytoma from the adrenal medulla. Both malignancies are rare. ACC has an estimated incidence of ∼0.5-2 new cases per million people per year.1,2 Phaeochromocytomas are catecholamine-producing neuroendocrine tumours arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia. The latter are usually called paraganglioma, leading to the combined term phaeochromocytomas and paragangliomas (PPGLs).
Topics & Concepts
MedicineAdrenocortical carcinomaClinical PracticeOncologyInternal medicineFamily medicineAdrenal and Paraganglionic TumorsCancer, Hypoxia, and MetabolismHormonal Regulation and Hypertension