Sclerosing Epithelioid Fibrosarcoma of the Spine: Diagnosis and Treatment of a Rare Entity

Sclerosing epithelioid fibrosarcoma (SEF) is a rare subtype of sarcoma with high rates of local recurrence and distant metastasis. Morphologically, it resembles other mesenchymal and non-mesenchymal tumors, making it a diagnostic challenge. Treatment relies mostly on surgery with adjuvant chemotherapy or radiotherapy (RT). A 46-year-old woman who presented with lumbar pain and weight loss underwent a computed tomography (CT) scan, magnetic resonance imaging (MRI), and a [18F]-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET/CT) scan, which showed a lesion involving the L5 vertebra. An incisional biopsy of the lesion established the diagnosis of SEF, with diffuse expression of MUC4 and focal expression of EMA. The patient was treated with neoadjuvant RT followed by surgery. Histology was congruent with the previous diagnosis and demonstrated post-radiation changes. In conclusion, SEF is an aggressive type of sarcoma that is easily misdiagnosed, so it is important to consider it in the differential diagnosis to avoid unbeneficial treatments and a detriment to patient survival.