Primary intraabdominal malignant mesenchymal tumors are very rare. There are just few cases of intraabdominal visceral malignant fibrous histiocytoma in the literature. We report a case of primary malignant fibrous histiocytoma of the spleen in a 57-year-old man, with a recurrence eight years after the splenectomy. After the initial surgery the patient was without complaints, and refused to receive chemotherapy or radiotherapy. Eight years after the surgery the patient reported due to general weakness and malaise when the diagnosis of disease relapse was established. Radical surgery was performed although the tumor involved large curvature of the stomach, left crus of the diaphragm, splenic flexure of the colon and tail of pancreas. Four months after the surgery patient died. To the best of our knowledge, to date, only 18 cases have been reported in the literature, describing malignant fibrous histiocytoma of the spleen.
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