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.
SeminarPhaeochromocytoma
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
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