CAJ | 학술논문

目的：探讨儿童原发性脊髓生殖细胞瘤的临床特点、诊断、治疗及预后。方法报道1例儿童原发于脊髓的混合性生殖细胞瘤，目前国内外未见此类报道，并对儿童原发性脊髓生殖细胞瘤的相关文献进行复习和分析。结果5岁男孩临床主要表现为臀部疼痛进行性加重，性早熟，血清及脑脊液甲胎蛋白、β-人绒毛膜促性腺激素明显升高，磁共振成像示L2-3水平椎管内占位，组织病理示椎管内混合性生殖细胞瘤（生殖细胞瘤＋畸胎瘤）。采用18F-脱氧葡萄糖正电子发射计算机断层显像可协助显示病灶，并通过监测最大标准摄取值的变化评估疗效及监测病情有无复发。该患儿放疗效果差，化疗效果尚好。结论儿童生殖细胞瘤原发于脊髓非常罕见，临床表现与累及的脊髓节段相一致，应当与原发于脊髓其他肿瘤相鉴别。单纯性儿童原发性脊髓生殖细胞瘤对放、化疗均敏感，预后较好。
목적：탐토인동원발성척수생식세포류적림상특점、진단、치료급예후。방법보도1례인동원발우척수적혼합성생식세포류，목전국내외미견차류보도，병대인동원발성척수생식세포류적상관문헌진행복습화분석。결과5세남해림상주요표현위둔부동통진행성가중，성조숙，혈청급뇌척액갑태단백、β-인융모막촉성선격소명현승고，자공진성상시L2-3수평추관내점위，조직병리시추관내혼합성생식세포류（생식세포류＋기태류）。채용18F-탈양포도당정전자발사계산궤단층현상가협조현시병조，병통과감측최대표준섭취치적변화평고료효급감측병정유무복발。해환인방료효과차，화료효과상호。결론인동생식세포류원발우척수비상한견，림상표현여루급적척수절단상일치，응당여원발우척수기타종류상감별。단순성인동원발성척수생식세포류대방、화료균민감，예후교호。
Objective To investigate the clinical features, diagnosis, treatment and prognosis of prima-ry intramedullary spinal cord germinoma ( PISCG) in children.Methods One child with primary intramedul-lary spinal cord mixed germinoma was reported in this article .To our knowledge , there has been no previous report of such cases at home and abroad .The related literature of PISCG was reviewed and analyzed .Results The clinical manifestations of the 5-year-old boy included the progression of hip pain and precocious puberty . Alpha-fetoprotein ( AFP) andβ-human chorionic gonadotrophin (β-HCG ) levels elevated obviously in serum and cerebrospinal fluid .Magnetic resonance imaging ( MRI) demonstrated an intraspinal mass extending from L2 to L3.Histopathological examination showed mixed germinoma (germinoma and teratoma).18F-fluorodeoxy-glucose positron emission tomography (18F-FDG-PET) was applied to help displaying the lesion, evaluating therapeutic effect and monitoring recurrence via the maximum standardized uptake value ( SUVmax) .The child responded poor to radiotherapy , while fair to chemotherapy .Conclusions PISCG in children is extremely rare.Its clinical manifestations are consistent with the involved segments of the spinal cord and should be dif-ferentiated from other primary tumors in spinal cord .Simple PISCG in children is sensitive to radiotherapy and chemotherapy , with generally favorable prognosis .