Feocromocitoma del órgano de Zuckerkandl. A propósito de un caso

Extraadrenal pheochromocytomae represent 17-18% of pheochromocytomae, 85% of which are located infradiaphragmaticaly and generally wherever there is cromaffin tissue, as in this case, located at the organ of Zuckerkand. They can also appear in the context of family syndromes, i.e. phacomatosis and MEN (IIA and IIB).The authors present a case of pheochromocytoma located in the organ of Zuckerkand, diagnosed in the course of uncontroled HBP. Diagnosis was achieved by metanephrine determinations and radiological tests (ultrasound, CAT and MIBG). This patient was surgically treated by exeresis of the lession previous alpha and beta adrenergic blockade with phenoxybenzamine and propanolol. Currently this patient is asymptomatic with normal blood pressor and metanephrines blood levels.Extraadrenal pheochromocytomae are usually located in superior paraaortic infradiaphragmatic region. The grade of malignancy must be established by histopathological findings, local recidives and metastasis. After clinical suspicion and the finding of a retroperitoneal mass with ultrasound, CAT and/or MRI, MIBG must be performed to confirm the diagnosis as well as to leave out possible tumours at distance. Treatment is surgery previous blockade of cathecolamines secretion by the preoperative administration of alpha blockers, with or without beta blockers.