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Phaeochromocytomas, paragangliomas, and neuroblastoma 

Phaeochromocytomas, paragangliomas, and neuroblastoma
Chapter:
Phaeochromocytomas, paragangliomas, and neuroblastoma
Author(s):

Isla S. Mackenzie

and Morris J. Brown

DOI:
10.1093/med/9780199235292.003.0554
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date: 26 June 2019

Phaeochromocytomas are rare neuroendocrine tumours of neural crest origin, which often produce excess catecholamines (1). Although usually arising from the chromaffin cells of the adrenal medulla, phaeochromocytomas may also arise at other sites of sympathetic or parasympathetic chromaffin tissue anywhere from the base of the skull to the pelvis. Extra-adrenal phaeochromocytomas are called paragangliomas. Some patients with phaeochromocytoma or paraganglioma present with the classical triad of symptoms of headaches, palpitations, and sweating but many others present with less specific features such as hypertension or with an unidentified mass lesion.

Owing to the rarity of the condition and the relatively nonspecific symptoms with which it often presents, it is not unusual for several years to pass from symptom onset until the diagnosis of phaeochromocytoma is made. However, the consequences of not finding a phaeochromocytoma can be severe and may even result in death. In fact, in one study, around 50% of cases of phaeochromocytoma found at post mortem were unsuspected during life. Interestingly, a former US President, Dwight Eisenhower, was found to have a 1.5 cm adrenal phaeochromocytoma at post mortem, which was undiagnosed during life despite a history of severe hypertension and headaches (2). Patients with untreated phaeochromocytoma are at risk of the cardiovascular consequences of catecholamine surges, including hypertensive emergencies, intracerebral haemorrhage, and acute heart failure. Approximately 10% of phaeochromocytomas are malignant and some represent part of familial syndromes. The genetic basis of many phaeochromocytomas is becoming increasingly apparent as more mutations are found.

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