Extra-adrenal phaeochromocytoma in a resource poor setting: A case report

Abstract

Abstract Phaeochromocytomas are catecholamine-secreting tumors arising in the chromaffin cells of the adrenal medulla. They are a rare cause of secondary hypertension. However, catecholamine secreting tumors may also be found in the extra-adrenal sites, producing similar symptoms as the adrenal phaeochromocytoma. The extra-adrenal phaeochromocytomas, are referred to as paragangliomas (PGLs). About 75% of extra-adrenal phaeochromocytomas are intra-abdominal, mostly located in perinephric, periaortic, and bladder regions. Most phaeochromocytomas secrete excessive amount of epinephrine and norepinephrine, whereas most paragangliomas secrete only norepinephrine. The excessive secretion of these products could lead to paroxysms of symptoms that could be life threatening. Medical management is initially offered, but definitive treatment involves surgical removal of the tumor, which requires promptness on the both the clinician and the patient sides. We present a case of an extra-adrenal phaeochromocytoma in an adult male with revealing imaging of a mass surrounding the bladder. The patient was managed with both alpha- and beta-adrenergic blockers. He declined the surgery and eventually died after appearing in an acute hypertensive crisis.