Ossifying Fibromyxoid tumor of soft parts in head and neck: case report and literature review

Abstract

BackgroundOssifying 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 presentationA 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.ConclusionsIt 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.

Ossifying fibromyxoid tumor in the lower extremity mimicking a sebaceous cyst. Case report and literature review

“…In terms of site, OFMT is common in the extremities [ 1 ], although it has also been reported in the soft parts of the head and neck [ 8 , 9 ]. As for presentation, OFMT presents as an asymptomatic slow growing deeply-seated mass attached to the surrounding structures such as fascia or skeletal muscles [ 7 ].…”

Ossifying fibromyxoid tumor of soft tissue: A case report with review of literature

“…It was firstly described in 1989 by Enzinger in a series of 59 cases from the Armed Forces Institute of Pathology (AFIP) [ 1 , 2 , 6 ]. Its pathogenesis is still unclear [ 7 ]. Rearrangement of the PHF1, locus at the chromosome 6p21 had recently been observed in 80% of OFT [ 1 , 3 ].…”

“…Rearrangement of the PHF1, locus at the chromosome 6p21 had recently been observed in 80% of OFT [ 1 , 3 ]. Graham had also reported a deletion of the SMARCB1/INI1, locus at the chromosome 22p12.1 in 71% of cases [ 1 , 7 , 8 ].…”

“…The OFT is a mesenchymal tumor of an uncertain differentiation. Cartilaginous, smooth muscle and nerve sheath origin was suggested but this theory needs to be proven [ 1 , 3 , 6 , 7 ].…”

“…It occurs usually in the extremities [ 1 , 6 ]. Less common sites include the trunk, the head and the neck, the oral cavity, the retroperitoneum and the mediastinum [ 1 , 3 , 6 , 7 ]. It is an indolent, well-defined, slow growing and firm mass located in the subcutaneous tissue [ 9 ].…”