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Primary Malignant Fibrous Histiocytoma of the Duodenum

Background/Aim: We report on a case of primary malignant fibrous histiocytoma of the duodenum. Methods: Report of a case and review of the literature on the occurrence of this tumor in the alimentary tract. Results: The neoplasm was detected incidentally at the time of diagnostic workup for upper gastrointestinal bleeding. Curative surgery using the Whipple procedure was carried out with uneventful postoperative course; no evidence of liver metastases was detected during intraoperative ultrasonography. The patient died of diffuse metastatic liver disease 2 months after surgical resection. Up-to-date review of the literature has added only 3 cases to those previously reported, including that reported by us. Conclusions: The biological behavior of malignat fibrous histiocytomas is extremely aggressive and mainly conditioned by size and histological grading. The treatment of choice, whenever possible, is based on early and complete surgical excision of the tumor.