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• Phaeochromocytomas are neuroendocrine tumours of the adrenal medulla

  • 10% multiple

  • 10% extra-adrenal

  • 10% malignant

• Extra-adrenal tumours are called paraganglionomas

• Most secrete adrenaline

• Some secrete noradrenaline and dopamine

• Clinical effects are due catecholamine excess

Clinical features

• Phaeochromocytomas account for 0.1% cases of hypertension

• Symptoms are often sporadic and paroxysmal

• Attacks may last minutes or hours and occur at variable intervals

• Include hypertension, palpitations, tachycardia and sweating

• 50% of patients develop chest pain

• Chronic effects include hypovolaemia and cardiomyopathy

• Phaeochromocytomas can be associated with:

  • Multiple endocrine neoplasia syndrome (Type 2)

  • Neurofibromatosis

  • Von Hippel Lindau syndrome

Investigations

• To confirm diagnosis need to demonstrate catecholamine excess with

  • 24-hour urinary vanniyl mandellic acid (VMA)

  • 24-hour urinary total catecholamines

  • Serum adrenaline or noradrenaline

• Tumour can be localised with:

  • Abdominal CT

  • MIBG (meta-iodobenzylguanidine) scanning

Picture provided by Suzanne Yoder, UCSD Medical Centre, San Diego, USA

Management

• Clinical features can not be controlled pharmacologically

• Need adrenalectomy after appropriate preoperative preparation

• Requires close cooperation between surgeon and anaesthetist

• Preoperative preparation requires:

  • Alpha-blockade with phenoxybenzamine for at least 2 weeks preoperatively

  • Volume expansion with intravenous fluids

  • Beta-blockade after alpha-blockade

  • Beta-blockade without alpha-blockade can cause a hypertensive crisis

• Intraoperative problems include:

  • Hypertension associated with handling of the tumour

  • Hypotension following devascularisation of the tumour

• Intraoperative blood pressure control achieved with fluids, nitroprusside and dopamine infusions

Picture provided by David Martin, Princess Alexandra Hospital, Brisbane, Australia

Bibliography

Lenders J W,  Eisenhofer G,  Mannelli M et al.  Phaeochromocytoma.  Lancet 2005;  366:  665-675.

Peplinski G R,  Norton J A.  The predictive value of diagnostic tests for phaeochromocytoma.  Surgery 1994;  116:  1101-1110.

Roman S.  Phaeochromocytoma and functional paraganglioma.  Curr Opin Oncol 2004;  16:  8-12.

Williams D T,  Dann S,  Wheeler M H.  Phaeochromocytoma - views on current management.  EJSO 2003;  29:  483-490.

Last updated: 05 January 2008

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