Elsevier

The Lancet

Volume 366, Issue 9486, 20–26 August 2005, Pages 665-675
The Lancet

Seminar
Phaeochromocytoma

https://doi.org/10.1016/S0140-6736(05)67139-5Get rights and content

Summary

Phaeochromocytomas are rare neuroendocrine tumours with a highly variable clinical presentation but most commonly presenting with episodes of headaches, sweating, palpitations, and hypertension. The serious and potentially lethal cardiovascular complications of these tumours are due to the potent effects of secreted catecholamines. Biochemical testing for phaeochromocytoma is indicated not only in symptomatic patients, but also in patients with adrenal incidentalomas or identified genetic predispositions (eg, multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1, and mutations of the succinate dehydrogenase genes). Imaging techniques such as CT or MRI and functional ligands such as 123I-MIBG are used to localise biochemically proven tumours. After the use of appropriate preoperative treatment to block the effects of secreted catecholamines, laparoscopic tumour removal is the preferred procedure. If removal of phaeochromocytoma is timely, prognosis is excellent. However, prognosis is poor in patients with metastases, which especially occur in patients with large, extra-adrenal tumours.

Section snippets

Genetics of phaeochromocytoma

Advances in genetics and recognition of the high prevalence of phaeochromocytoma in certain familial syndromes led to mandatory routine screening in patients with identified mutations, even in the absence of typical clinical signs and symptoms.40 Accumulating evidence also suggests that a hereditary basis for phaeochromocytoma is more frequent than previously thought, accounting for up to 24% of patients with the tumour with no obvious initial evidence of a syndrome or family history.13, 24, 41

Biochemical testing

All patients with suspected phaeochromocytoma should undergo biochemical testing. These include patients with paroxysmal signs or symptoms suggestive of a phaeochromocytoma; patients with recent, therapy-resistant, or volatile hypertension; patients with a paradoxical blood pressure response during surgery and anaesthesia; patients with a hereditary predisposition for a phaeochromocytoma; asymptomatic patients with an adrenal incidentaloma; and patients with sudden attacks of anxiety. Because

Preoperative management

Once a phaeochromocytoma is located, complications during surgery need to be kept to a minimum by appropriate preoperative medical treatment. With adequate pretreatment, perioperative mortality has fallen to less than 3%, which emphasises the importance for adequate preoperative management.12, 88, 89 The major aim of medical pretreatment is to prevent catecholamine-induced, serious, and potentially life-threatening complications during surgery, including hypertensive crises, cardiac

Search strategy and selection criteria

We searched the databases PubMed and EMBASE (Jan 1, 2000, to May 1, 2005) using the following keywords: “phaeochromocytoma”, “paraganglioma”, and “extra-adrenal”. We also included review articles, book chapters, or commonly referenced older publications. We reviewed the reference lists of articles identified by the search strategy and selected those we judged relevant. The search was restricted to papers published in English.

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