Sclerosing Epithelioid Fibrosarcoma; A Case report and review of literature

Abstract

Abstract Introduction/Objective Sclerosing Epithelioid Fibrosarcoma (SEF) is an unusual, rare clinically aggressive form of soft tissue sarcoma. It is characterized by a slow evolution, with local recurrences and late metastases that are mainly pulmonary and pleural in about 80% of cases. SEF has distinctive morphology and occurs most commonly in deep soft tissue of adult extremities. Lesions involving the head and neck region are uncommon and rare intraosseously in oral cavity. Methods/Case Report Herein we report a case of a 52-year-old male who presented with a symptomatic radiolucent lesion at apex #18, with clinical impression of periapical granuloma. The patient did present with pain associated with lower left quadrant #18 area, for several days to pressure and hot temperature. A periapical radiograph revealed a large ill-defined radiolucent area at apex of resorbing roots #18. The patient’s medical history was significant with history of cancer diagnosis of sclerosing epithelioid fibrosarcoma of skull treated by chemotherapy and radiation. Microscopic examination of the specimen revealed a multi-fragmented specimen consisting of numerous fragments and islands of highly cellular, basophilic bone and osteoid surrounded by loose fibrous stroma which contains large lobules and islands of round to oval cells, with distinct cell borders and faintly granular eosinophilic cytoplasm. Separate islands of tumor cells surround large islands of necrosis with the background stroma appearing as hyalinized and eosinophilic with the basaloid cells demonstrating smudge and crush artifact. Peripherally, spindled cells are noted and faint areas of eosinophilic osteoid within the eosinophilic background stroma. Lesional cells are MUC4 strong, diffusely positive and strongly positive for INI-1. CD43, CD20, PAX-5, CD3, Desmin, CD34, S100, CD99, AE1/3 and SMA are interpreted to be negative. A diagnosis of metastatic sclerosing epithelioid fibrosarcoma was made, with correlation with the primary lesion was recommended. Results (if a Case Study enter NA) N/A Conclusion The clinical and histological findings of our case correlate with the diagnostic criteria of sclerosing epithelioid fibrosarcoma. Despite its microscopic features can create diagnostic difficulties, in that SEF can resemble a variety of benign and pseudosarcomatous as well as malignant lesions, our case diagnosis was confirmed by morphology and MUC4 diffuse strong reactivity and negativity for other markers.