Phaeochromocytoma

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 ef...

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Published in:The Lancet (British edition) 2005-08, Vol.366 (9486), p.665-675
Main Authors: Lenders, Jacques WM, Eisenhofer, Graeme, Mannelli, Massimo, Pacak, Karel
Format: Article
Language:English
Subjects:
Adrenal glands
Adrenals. Adrenal axis. Renin-angiotensin system (diseases)
Biological and medical sciences
Diagnostic tests
Endocrinopathies
General aspects
Genetic screening
Genetics
Humans
Hypertension
Medical sciences
Medical treatment
Mutation
Non tumoral diseases. Target tissue resistance. Benign neoplasms
Pathology
Pheochromocytoma - diagnosis
Pheochromocytoma - genetics
Pheochromocytoma - therapy
Surgery
Thyroid
Tumors
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container_title The Lancet (British edition)
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creator Lenders, Jacques WM
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description 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.
doi_str_mv 10.1016/S0140-6736(05)67139-5
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subjects Adrenal glands
Adrenals. Adrenal axis. Renin-angiotensin system (diseases)
Biological and medical sciences
Diagnostic tests
Endocrinopathies
General aspects
Genetic screening
Genetics
Humans
Hypertension
Medical sciences
Medical treatment
Mutation
Non tumoral diseases. Target tissue resistance. Benign neoplasms
Pathology
Pheochromocytoma - diagnosis
Pheochromocytoma - genetics
Pheochromocytoma - therapy
Surgery
Thyroid
Tumors
title Phaeochromocytoma
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