This paper reports a very unusual site of ossifying fibromyxoid tumor (OFMT) and review the medical literature. In the maxillofacial region, OFMT has been rarely reported. However, malignant OFMT was recently traced in the tongue. Clinicians and maxillofacial pathologists should pay a rapt attention to such an innocent looking lesion that can be easily confused with the ossifying fibrous epulis. This holds true when OFMT is encountered in the gnathions.Keywords: Ossifying Fibromyxoid Tumor; Gnathic Lesions; Fibro-Osseous Lesions and trabeculae of ovoid cells swimming in a fibromyxoid stroma. OFMT was classified as "typical" and "atypical". Histologically, the presence of high cellularity or high nuclear grade, with mitotic activity >2 mitotic figures per 50 high power fields revealed a statistically significant potential for a malignant behavior. This find is characteristic of the so-called "atypical" OFMT. It proved not to be only potentially malignant, but also revealed 60% local recurrence rate and 60% metastasis incidence. Even in typical OFMT cases, the recurrence and metastatic rates, 17% and 5%, respectively, make it a lesion of intermediate malignancy. There are no specific immunohistochemical markers for OFMT. It shows a positive reactivity, sometimes, for S-100 and other bone markers [8].
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