Abstract

ABSTRACTWe report the case of a rare lipoma arising in the epidural space of a 14-year-old boy without spinal dysraphism. Lipomas are rare in pediatric soft tissue tumors, accounting for only about 4% of cases. The incidence of an intraspinal epidural lipoma without spinal dysraphism is extremely rare in pediatric patients. In this case, the patient had progressive motor deficits in the lower extremities and difficulty in urination and defecation. Magnetic resonance imaging showed an extradural tumor compressing the spinal cord at the T3–T7 level. Because of the progressive neurological deficits, we performed an emergency surgery. The tumor was completely resected en bloc, and histopathology revealed mature adipose tissue with fibrous septa, diagnosed as atypical lipomatous tumor / well-differentiated liposarcoma. The patient fully recovered and there was no tumor recurrence for 6 years since the surgery. However, re-examination using fluorescence in situ hybridization after 6 years of surgery changed the diagnosis to lipoma as no amplification of murine double-minute type 2 oncogene was observed. In liposarcoma, histopathological diagnosis using fluorescence in situ hybridization is mandatory. Our case illustrates that immunohistochemical diagnosis alone can be misleading. Hence, prompt surgery is required for progressive neuropathy.

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