Malignant fibrous histiocytoma of the pancreas.

Abstract Malignant fibrous histiocytoma (MFH) is a well-recognized soft-tissue sarcoma; however, its presentation as a primary neoplasm of the pancreas is unusual. We report a rare case of primary MFH of the pancreas treated with wide local excision and postoperative chemotherapy. Three cases of MFH of the pancreas have previously been reported in the world literature. The natural history of MFH of the pancreas cannot be summarized due to the rarity of the tumor. It appears, however, that the behavior of this tumor parallels the behavior of retroperitoneal MFH in terms of aggressiveness and poor prognosis.