Pheochromocytoma and paraganglioma

Pheochromocytomas and paragangliomas belong to the rare neuroendocrine tumors. These tumors are characterised by their catecholamine secretion and most of the symptoms are related to their hormonal activity. In case of clinical suspicion, biochemical evaluation of the excessive catecholamines and their metabolites is the cornerstone of the diagnosis. Regarding imaging, computer tomography is preferred, that should be performed for localisation and staging of the tumor. Functional imaging is also essential for the evaluation of the disease, and helpful for the treatment planning of the metastatic disease. For localised tumors, surgery is the preferred treatment option. In case of metastatic disease, the choice of therapy is similar to that used in case of other neuroendocrine tumors. The measurement of progression, the presence of symptoms as well as the tumor load are the most important informations for treatment decision. Systemic therapy options include radiometabolic therapy, chemotherapy and targeted agents.