[Case of recurrent retroperitoneal malignant paraganglioma 25 years after surgery]

A 41-year-old man was referred to our hospital for a left renal mass detected by screening computed tomography for hypertension. He had undergone surgery for left pheochromocytoma at 16 years of age in 1981, and the last follow-up was in 1986. Although a 131I-metaiodebenzylguanidine (MIBG) scintigram did not show abnormal uptake, elevated levels of serum cathecholamines were demonstrated. The left renal mass positioned in the upper pole and inside of the kidney had invaded the pancreas, colon and spleen. Surgical resection was done under a diagnosis of recurrent pheochromocytoma, and the tumor was removed with other organs, including normal left adrenal gland that was confirmed histologically. Histologically and immunohistochemically, the resectioned tumor was invasive malignant paraganglioma and diagnosed as the local recurrence or metastasis of previously resectioned retroperitoneal paraganglioma, which was believed to have grown slowly.