Metastatic Sclerosing Epithelioid Fibrosarcoma at Diagnosis: A Case Report

Sclerosing epithelioid fibrosarcoma (SEF) is a rare and aggressive neoplasm composed of epithelioid cells arranged in strands and nests embedded in a highly sclerotic collagenous stroma. We report a case of a 36-year-old man who started with lumbar pain, with extension to both legs, night sweats, and weight loss. He underwent magnetic resonance imaging (MRI) of the lumbar spine; computed tomography (CT) scan of the chest, abdomen, and pelvis; and [18F]-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) scan. The CT scan revealed a 13 cm thoracic mass, the MRI presented with diffuse neoplastic invasion of the vertebrae, and the PET showed hepatic, bone, and possibly pulmonary metastases. The histological diagnosis was compatible with SEF. The disease progressed very quickly, namely, with an episode of spinal cord compression, which made the patient paraplegic. He underwent surgery and, subsequently, radiotherapy (RT). Due to the clinical and analytical evolution, it was not possible to initiate systemic treatment and the patient ultimately passed away. In conclusion, SEF is an aggressive type of sarcoma that affects middle-aged patients, with high rates of distant metastases and mortality. The usual treatment is surgery followed by either radiotherapy or chemotherapy. However, further clinical trials are needed to find more systemic target therapies.