Malignant fibrous histiocytoma of the spleen: an extremely rare entity.

Primary malignant fibrous histiocytoma (MFH) of the spleen is extremely rare. Since the first description of primary splenic MFH reported by Govoni et al in 1982, to the best of our knowledge, only twelve cases of MFH of the spleen have been reported in the literature. We herein report a rare case of primary splenic MFH in a 30-year-old Indian male who presented with abdominal pain with a history of recurrent hydatid cyst of liver and spleen. A computed tomography (CT) scan was performed and a diagnosis of splenic hydatid cyst was made. Splenectomy was done. On histopathological examination, a diagnosis of malignant mesenchymal tumor, possibly storiform variant of malignant fibrous histiocytoma, was made. On immunohistochemistry, the tumor was positive for vimentin and CD68. The post operative period was uneventful. Compared with the twelve previously cases of MFH of the spleen, our patient is the youngest case reported so far.