Symptoms and treatment of Phaeochromocytoma in MEN 2b

The diagnosis and management of phaeochromocytomas in MEN2b is essentially the same as in MEN2a.

Phaeochromocytomas are tumours of the medulla of the adrenal glands which secrete the catecholamines, mainly adrenaline and noradrenaline, occurring in 50% of MEN 2b patients. Individuals may be asymptomatic and the tumour detected during screening of the urinary catecholamines (see 'Family Screening' section). Alternatively signs and symptoms such as intermittent high blood pressure, headaches and palpitations and sweating may occur.

Investigation of phaeochromocytoma in MEN is the same as in the sporadic form. However, differences in the two forms include a greatly increased incidence of bilateral tumours in the MEN form versus the sporadic form (70% cases versus 10%) and the very rare occurrence of tumours outside the adrenal in the MEN form compared to the 10% incidence for the sporadic form.

The recommended treatment for phaeochromocytoma in MEN 2 is therefore bilateral adrenalectomy (removal of both adrenal glands) due to the fact that the tumour commonly arises in both glands. This necessitates lifelong glucocorticoid and mineralocorticoid replacement (hormones produced by the unaffected adrenal cortex which is also removed at operation).

For much more information on phaeochromocytoma and adrenal gland physiology please see the relevant parts of the 'Adrenal Gland' section of this website.