Thoracic Spinal Sclerosing Epithelioid Fibrosarcoma Mimicking Schwannoma: Case Report and Literature Review.

BACKGROUND/

OBJECTIVES

Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue sarcoma with high rates of local recurrence and distant metastasis. Primary spinal involvement is exceedingly uncommon and often misdiagnosed due to radiological and histopathological resemblance to more frequent spinal tumors. The objective of this study is to present a rare case of thoracic spinal SEF and to contextualize it within the available literature.

METHODS

We describe the case of a 37-year-old woman presenting with progressive back pain and dysesthesia. MRI demonstrated a heterogeneously enhancing mass at the left T10-T11 neural foramen, initially interpreted as a common nerve sheath tumor. Gross total resection (GTR) was achieved, and histopathological analysis revealed a SEF. Clinical course, adjuvant therapies, and outcomes were evaluated, together with a review of previously reported spinal SEF cases.

RESULTS

Despite GTR followed by adjuvant chemotherapy, local recurrence occurred 18 months later. The patient underwent subtotal resection (STR) with adjuvant proton therapy. At 18-month follow-up after the second procedure, she remained neurologically stable and disease-free. The literature review confirmed the rarity of spinal SEF, its frequent misdiagnosis, and the absence of standardized therapeutic protocols.

CONCLUSIONS

Spinal SEF is a rare malignancy that can mimic benign spinal tumors, delaying diagnosis. Its management relies on multidisciplinary assessment, individualized therapy, and long-term follow-up. This report increases awareness of spinal SEF and provides additional evidence to support clinical decision-making in rare spinal tumors.