Abstract

Benign fibrous histiocytoma (BFH) is a mesenchymal tumor usually constituted of spindle and epithelioid cells, which occurs predominantly in the bone and soft tissues. Histopathologically, it reveals 2 types of cells, fibroblasts and histiocytes, arranged in storiform pattern and interlacing fascicles without nuclear pleomorphism, necrosis, and low mitotic activity. The granular cell variant of BFH is extremely rare. A 64-year-old woman was referred presenting an osteolytic lesion in the anterior region of the maxilla, mimicking nasopalatine duct cyst. After excisional biopsy, microscopy revealed a storiform arrangement of spindle to epithelioid cells, most of them with PAS-D–positive granular cytoplasm, surrounded by sclerotic fibrous stroma. Immunohistochemistry showed positivity for vimentin, CD68, CD163, α–smooth muscle actin, and focal FXIIIA. Ki-67 was

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