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Phaeochromocytomas are functional tumours that arise from chromaffin cells
in the adrenal medulla.
- incidence among the general population is about 0.8 per 100,000 person-years,
and is estimated to be 0.1-0.6% in the hypertensive population
- diagnosis usually takes place in patients aged 40-50 years
- however, hereditary variants, such as multiple endocrine neoplasia type
2, Von Hippel-Lindau disease, neurofibromatosis type 1 and the pheochromocytoma-paraganglioma
syndrome, can present earlier
Phaeochromocytomas usually secrete a combination of noradrenaline and adrenaline,
but some tumours may also secrete dopamine and rarely ACTH causing Cushing's syndrome.
- rare tumours of (APUD cells)
- chromaffin cells of the adrenal medulla
- paraganglion cells of the sympathetic nervous system
- APUD cells : embryologically derived from the neuroectoderm
- functionally involved in amine and amine precursor uptake and decarboxylation
- occur in a variety of non endocrine (intestines, lungs) and endocrine (adrenals,
thyroid, parathyroid) tissues
- paragangliomas (Extra-adrenal phaeochromocytomas), also called the vascular
head and neck tumors, most commonly found at the carotid bifurcation
- chromaffin tumours arising from aorticosympathetic paraganglia have
histologic, biochemical and clinical similarities to phaeochromocytomas
and are sometimes referred to as extra-adrenal phaeochromocytomas
May be discovered as an adrenal "incidentaloma"( 5% are pheochromocytomas).
- Harmut PH et al. Pheochromocytomas, multiple endocrine neoplasia type 2,
and von Hippel-Lindau disease. NEJM 1993;329 (21): 1531-8.
- Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet.
- Guerrero MA, Schreinemakers JM, Vriens MR, et al. Clinical spectrum of pheochromocytoma.
J Am Coll Surg. 2009;209:727-32.
Last reviewed 01/2018