Malignant giant pheochromocytoma: a case report and review of the literature

Abstract

Malignant pheochromocytoma is a rare disease and surgical resection is the only curative treatment. There are no definitive histological or cytological criteria of malignancy, as it is impossible to determine this condition in the absence of advanced locoregional disease or metastases. We report a case of a patient with a giant retroperitoneal tumour, the second largest to be published, which was diagnosed as a malignant pheochromocytoma; it was treated with surgery. The literature is reviewed to evaluate tumour features and criteria to distinguish between benign and malignant pheochromocytomas.

Fig. 1.

Computed tomography scan of the left suprarenal vein thrombus.

Fig. 2.

Arteriography image. Tumour vascularization from the left kidney, superior mesenteric and splenic arteries.

Fig. 3.

Intraoperative image where the mass is seen behind the stomach and pancreatic body and tail. Notice the great vascularization.

Fig. 4.

Resection specimen: tumour, spleen, pancreatic body and tail.