Guillain-Barré 증후군 양상으로 발병한 원발성 척추내 말초성 원시신경외배엽종양 1례

A Case of Primary Intraspinal Peripheral Primitive Neuroectodermal Tumor (pPNET) Mimicking Guillain-Barré Syndrome

Primary intraspinal peripheral primitive neuroectodermal tumors (pPNET) are ex- tremely rare. We report a 10-month-old girl presented with rapidly progressive pa- raparesis and raised cerebrospinal fluid protein. A magnetic resonance image demon- strated an intramedullary mass from C3 to T3 level and intradural extramedullary nodules in the lumbosacral area. Surgery was performed with partial tumor removal and histologic examination revealed a small round cell tumor and immunohistoche- mical characteristics of pPNET. She died due to tumor progression at four months after initial diagnosis. This case has been reported to raise awareness among clini- cians to include the possibility of intraspinal tumors in the differential diagnosis of progressive neurological deficits mimicking Guillain-Barré syndrome.