Background
Pheochromocytomas are catecholamine producing tumors of chromaffin cells, that can occur sporadically or as part of a familial syndrome. Ten to 15 % of patients with pheochromocytoma develop malignant disease, which has an overall 5-year survival rate of 50%. There are currently no effective treatments for malignant pheochromocytoma, nor reliable pathological methods for distinguishing a benign from a malignant tumor or assessing potential for malignancy. The rarity of pheochromocytoma and the resulting fragmented nature of studies, typically involving small numbers of patients, represent limiting factors to the development of effective treatments and diagnostic or prognostic markers for malignant disease. 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 specialist centers.
