[Germline mutations define the prognosis and therapy of pheochromocytomas and paragangliomas].

Abstract

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, where the genetic background is of particular importance in terms of the diagnosis, follow-up, treatment and prognosis of the disease. Although the prognosis in benign cases is encouraging, in the case of malignant disease the mortality rates are dramatically worse due to the high tumor burden and possible cardiovascular complications caused by catecholamine oversecretion. In recent years, significant progress has been made both in the functional imaging of the disease and in the therapy of malignant diseases, which in many cases are also related to the underlying genetic background. The aim of our brief summary is to place the diverse genetic background and tumor biology of the disease in the context of diagnostics and therapy, thus highlighting the importance of our understanding of the disease at the individual patient level.