Maxilla tuberosity malignant fibrous histiocytoma with giant fibroblastic cells: Case report and review of literature

SummaryMalignant fibrous histiocytoma (MFH) of the maxilla is a rare neoplasm. A round 61 cases reported in the international literature since 1974. We present a rare case of primary MFH of the maxilla in the unusual location of maxilla in a 64-year-old man. The tumor was located in the left tuberosity of maxilla extending from the junction between soft and hard palate towards premolar area of edentulous ridge, and measured 7 cm × 6 cm. Histologically, it consisted of spindle-shaped, pleomorphic malignant cells in a storiform pattern associated with histiocyte-like cells and giant cells. Mitotic figures were frequent Immunohistochemically, most of the tumor cells were strongly positive for vimentin, and negative with S-100 protein, cytokeratin, actin, desmin, HMB45 and epithelial membrane antigen. Ultra structurally, the tumor have clearly shown spindle shaped fibroblastic and giant cells with well-known pleomorphic multi-segmented nuclei, prominent branching and often dilated rough endoplasmic reticulum (RER). Histopathological and ultra structural findings are consistent with high-grade MFH of the storiform/pleomorphic subtype. Four months later the patient came with residual/recurrent tumor that was confirmed histopathologically. The literature is briefly reviewed.