Phaeochromocytoma

Key Information

Appropriate Tests

 

A phaeochromocytoma is a catecholamine secreting tumour of the chromaffin cells from the adrenal medulla. A paraganglionoma is a catecholamine secreting tumour of chromaffin secreting cells from the sympathetic ganglia. These tumours are relatively rare with a prevalence of approximately 5 per million overall; ~0.5% of individuals with hypertension or ~5% of adrenal adenomas. They may also be familial occurring in Multiple endocrine neoplasia 2, Neurofibromatosis 2 and von Hippel-Lindau syndrome.

Initial screening for Phaeochromocytoma should include measurements of Free metanephrines and/or fractionated Metanephrines urine. Patient preparation is important and drug interferences should be considered when interpreting the results as they may cause false positive increases.

If indicated - Clonidine suppression test may be performed to further investigate borderline increases in plasma normetanephrine. Urine catecholamine measurements are less sensitive and not recommended.

If phaeochromocytoma is suspected, fine needle aspiration biopsy is contraindicated.

 

See also Multiple endocrine neoplasia (types 2A and 2B)

References: 

Eisenhofer G, Peitzsch M. Clin Chem 2014; 60: 1486-1499.

Eisenhofer G et al. Pharmacol Rev 2004; 5656: 331-349.