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Journal of the Korean Child Neurology Society 2010;18(2):326-331.
Published online November 30, 2010.
A Case of Primary Intraspinal Peripheral Primitive Neuroectodermal Tumor (pPNET) Mimicking Guillain-Barre Syndrome.
Jeong Yong Lee, Eun Hye Lee, Min Hee Jeong, Mi Sun Yum, Ho Joon Im, Jong Jin Seo, Young Shin Ra, Tae Sung Ko
1Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Korea. tsko@amc.seoul.kr
2Department of Neurosurgery, Asan Medical Center, University of Ulsan College of Medicine, Korea.
3Department of Pediatrics, Gangneung Asan Hospital, Korea.
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-Barre syndrome.
Key Words: Guillain-Barre Syndrome, Neuroectodermal Tumors, Primitive, Peripheral
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