Phaeochromocytomas and functional paragangliomas: Clinical management

Phaeochromocytomas (PH) and functional paragangliomas (FPGL) are neoplasms of adrenal (PH) or extra-adrenal (FPGL) chromaffin tissue that synthesize catecholamines. Catecholamines are converted into inactive metabolites, metanephrines, within the tumour and the diagnosis of PH/FPGL is therefore based on the quantification of plasma or urinary metanephrines. The tumour can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Patients are treated by tumour resection following alpha-blockade. PH and FPGL may be sporadic or part of several genetic diseases. Patients with PH/FPGL should be followed up indefinitely as the disease may recur, particularly if they have inherited or extra-adrenal tumours. About 10% of tumours are malignant either at initial surgery or during follow-up. Recurrences and malignancy are more frequent in cases with large or extra-adrenal tumours, and in SDHB mutation carriers. Treatments for progressive malignant PH/FPGL include tumour debulking, metabolic radiotherapy, chemotherapy, and possibly tyrosine kinase inhibitors.