Introduction and importance: Sclerosing epitheloid fibrosarcoma is a rare variant of low grade sarcoma with specific histologic and immunohistochemical features, and often associated with a poor prognosis.
Clinical presentation: We report a case of a 35-year old male who presented with 2 year history of lower back pain, radiating to both lower limbs, weakness to both lower limbs and weight loss more than 5kgs in the past 6 months, no past history of trauma, drenching night sweats, no TB contact history, no incontinence and no any comorbidities. He reported no history of alcohol intake or cigarette smoking, no history of exposure to radiations or similar presentation to his family and no features suggestive of metastasis. On examination - he had maximum midline tenderness L3-S1, no gibbous, upper limbs neurologically intact and power 3/5 to both lower limbs, sensation intact. Computed tomography scan and magnetic resonance imaging revealed an ill-defined osteolytic mass spanning L3 to L5, vertebral plana of L4 and involvement of the left paraspinal muscles. The tumor extended to the extradural space and was also abutting on the distal great vessels. Our intervention entailed laminectomy, tumor debulking and posterior instrumental fusion of the spine. Histopathology findings were in keeping with an invasive sclerosing epitheloid fibrosarcoma. He had adjuvant chemotherapy with good outcome.
Discussion: This case was unique due to its lumbar column location, abutting the distal vessels and despite its low grade, it illustrates the malignant potential which responded well to adjuvant chemotherapy.
Conclusion: Invasive SEF is an aggressive tumor that requires early diagnosis. Multi-modal treatment with surgical resection, adjuvant chemotherapy can improve patient survival and quality of life.
Keywords: Chemotherapy; Lumbar spine; Sclerosing epithelioid fibrosarcoma; Tumor debulking.
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