ABSTRACT
BACKGROUND: Ossifying fibromyxoid tumor of soft parts (OFMT), is a rare but morphologically distinctive neoplasm of uncertain histogenesis that most frequently affects middle-aged male adults. Clinically, it usually presents as a slowly enlarging, small, circumscribed mass, which in most cases is painless. OFMT is most frequently found within the subcutaneous tissues of extremities or trunk, and rarely in the oral/head and neck region. We present an unusual case of this tumor in the submandibular region, and, based on the current medical literatures this is probably the first case described in this anatomical location. CASE PRESENTATION: A 32-year-old male presented to our outpatient clinic with a right submandibular mass with 1-year of evolution. Excisional biopsy showed that it was characterized by ossification along the periphery of the lesion. The neoplastic cells were spindle-like with scant eosinophilic cytoplasm. These cells were arranged with uniform cell-to-cell space in a fibromyxoid stroma. Small and large clusters of calcifications were present within the tumor. Immunohistochemically, the case showed positive staining of S-100 protein, vimentin, nestin, calponin, SMA, GFAF, desmin, INI-1, caldesmon, and CD34. It also showed negative staining of CK, CK7, CK8/18, NF, and EMA. About 2% of neoplastic cells showed positive staining of Ki67. Based on these features, the final pathological diagnosis was OFMT. CONCLUSIONS: It is hoped that a greater understanding of OFMT in the head and neck region will avoid potential misdiagnosis, and contribute to determining the correct management, which appears to be complete surgical excision with close follow-up for recurrence surveillance.