RESUMO
A 45-year old woman presented with a recent paroxysmal hypertension and a flush syndrome. Physical examination revealed an abdominal mass with positive left lumbar contact. The urine metanephrine was high. The CT scan and the Magnetic Resonance imaging [MRI] showed a 10 cm retgroperitoneal tumour apart from the left adrenal gland and driving back the left kidney. The diagnosis of ectopic pheochromocytoma was suspected and the patient was operated. A tumour resection widened to the left adrenal gland and to the kidney was performed The specimen histology showed a well-differentiated liposarcoma associated to an adrenal medullary hyperplasia [AMH] Postoperatively blood pressure was stabilizsed at normal levels. The review of literature show that AMH mimicking a pheochromocytoma is rare and its association with a giant retroperitoneal liposarcoma has never been reported before