Management of Phaeochromocytoma and Paraganglioma

Abstract

Phaeochromocytomas and paragangliomas (PPGLs) are rare catecholamine producing tumours. The pathophysiology of PPGL is largely driven by genomic alterations. At least 35% of PPGLs occur as part of a hereditary syndrome. Catecholamine excess may lead to severe hypertension and potentially lethal cardiovascular complications. Therefore, prompt diagnosis is essential for effective treatment, usually by surgical resection. Biochemical testing should include analysis of plasma-free metanephrines or urinary fractionated metanephrines. Additional testing using the clonidine-suppression test is indicated in case of mildly elevated test results that cannot be explained by faulty sampling conditions, patient preparation, or by use of interfering medication. Following a biochemical diagnosis of PPGL, tumour localization can be achieved by CT or MRI. This chapter describes the imaging and treatment modalities that can be employed in the effective management of phaeochromocytoma and paraganglioma.