What are pheochromocytomas?

Pheochromocytomas are catecholamine-producing tumors formed by chromaffin cells in the adrenal gland that can occur sporadically or as part of a familial syndrome. Pheochromocytomas can also arise from tissues outside the adrenal glands, in which case they are also referred to as paragangliomas. Fifteen to seventeen percent of patients with these tumors have malignant disease, which has overall 5-year survival rates ranging from 50% to 60%. No curative treatments for malignant pheochromocytoma are available, nor are any reliable pathological methods for distinguishing benign and malignant tumors or assessing the potential for malignancy. The rarity of pheochromocytoma and resulting fragmented nature of studies of this tumor, typically involving small numbers of patients, limit the development of effective treatments and diagnostic or prognostic markers for malignant pheochromocytoma. Such development can be facilitated by the availability of new genomics- and proteomics-based tools, but this ideally requires comprehensive clinical studies involving large numbers of patients, stringently collected clinical data, tumor and blood samples, and interdisciplinary collaborations among multiple specialized centers.

Facts in brief