Endocrinology & Metabolic

Phaeochromocytoma

A catecholamine-secreting tumour of adrenal chromaffin tissue causing episodic or sustained catecholamine excess, with headache, palpitations, sweating and potentially life-threatening cardiovascular instability.

Definition

Phaeochromocytoma is a catecholamine-secreting tumour arising from adrenal medullary chromaffin cells; a related extra-adrenal tumour is a paraganglioma. PPGL may cause episodic or sustained catecholamine excess, or be discovered incidentally.

First principles

Phaeochromocytoma is a tumour of catecholamine-producing chromaffin cells

Chromaffin cells of the adrenal medulla release catecholamines. A phaeochromocytoma arises from adrenal chromaffin tissue; a paraganglioma is the extra-adrenal counterpart. Secretion may be episodic, sustained or clinically silent, so a patient can have attacks, persistent hypertension or an incidental adrenal mass.

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