Guillain-Barré 증후군 양상으로 발병한 원발성 척추내 말초성 원시신경외배엽종양 1례
A Case of Primary Intraspinal Peripheral Primitive Neuroectodermal Tumor (pPNET) Mimicking Guillain-Barré Syndrome
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참고문헌
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초록
저자들은 이전까지 특별한 문제 없이 지내다 빠르게 진행하는 사지 불완전 마비를 주소로 내원한 10개월 영아에서 Guillain-Barré 증후군 의심하에 면역글로불린 투여하였으나 증상 호전 되지 않았고, 이후 시행한 영상의학 및 조직학적 검사상 원발성으로 척추내 발생한 말초성 원시신경외배엽종양이 진단되었기에 이를 문헌 고찰과 함께 보고하는 바이다.
Abstract
Primary intraspinal peripheral primitive neuroectodermal tumors (pPNET) are extremely rare. We report a 10-month-old girl presented with rapidly progressive paraparesis and raised cerebrospinal fluid protein. A magnetic resonance image demonstrated 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 immunohistochemical characteristics of pPNET. She died due to tumor progression at four months after initial diagnosis. This case has been reported to raise awareness among clinicians to include the possibility of intraspinal tumors in the differential diagnosis of progressive neurological deficits mimicking Guillain-Barré syndrome.
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