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Primary Intramedullary Spinal Cord Mixed Germinoma in Children: Report of One Case and Review of Literature
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摘要:
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。 Abstract:Objective To investigate the clinical features, diagnosis, treatment and prognosis of primary intramedullary spinal cord germinoma(PISCG) in children. Methods One child with primary intramedullary 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) and β-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 differentiated from other primary tumors in spinal cord. Simple PISCG in children is sensitive to radiotherapy and chemotherapy, with generally favorable prognosis. -
Key words:
- spinal cord /
- mixed germinoma /
- children
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图 1 患儿腰椎MRI及18F-FDG-PET图像(箭头示病灶)
A.化疗前腰椎MRI矢状位增强T1WI示L1-2椎管内类圆形混杂信号影，实性部分及囊壁强化呈高信号，实性部分大小2.05 cm×1.07 cm×0.87 cm；B.第4程化疗后腰椎MRI矢状位增强T1WI示L1-2椎管内类圆形混杂信号影，实性部分较化疗前减小，实性部分大小1.97 cm×1.03 cm×0.76 cm；C.化疗前18F-FDG-PET融合图像示L1-2脊髓代谢增高灶SUVmax 3.4，S2水平代谢增高灶SUVmax 3.3；D.第4程化疗后18F-FDG-PET融合图像示L1-2脊髓代谢增高灶SUVmax 2.5，S2水平代谢增高灶SUVmax 2.5 MRI：磁共振成像；18F-FDG-PET：18F-脱氧葡萄糖正电子发射计算机断层显像；T1WI：T1加权像；SUVmax：最大标准摄取值
表 1 11例儿童原发性脊髓生殖细胞瘤的临床资料
病例 文献来源 性别 年龄
(岁)部位 手术方式 临床表现 病理学 治疗 随访 1 Hisa等(1985)[4] 男 5 T11-L3 活检 下肢疼痛、性早熟，血β-HCG 38~
92.9 U/L，CSF β-HCG 12 630 U/L生殖细胞瘤含STB 放疗+化疗 1年后复发 2 Slagel等(1995)[5] 女 16 T10-L5 部分切除 双髋疼痛，下肢疼痛，尿失禁，曾误
诊为转移性癌生殖细胞瘤 放疗 28年无复发 3 高玉平等(2002)[6] 男 7 T12-L4 全切 双下肢疼痛、无力，排尿困难，血
β-HCG 13 900 U/L生殖细胞瘤含STB 放疗 1年无复发 4 Chute等(2003)[7] 男 18 T7-T8 活检 踝关节无力，足下垂，2年后出现失
禁，影像学改变，CSF β-HCG 132 U/L生殖细胞瘤无STB 放疗 6个月无复发 5 Huang等(2004)[8] 男 18 C3-C6 部分切除 颈痛，双手感觉异常、无力 生殖细胞瘤 放疗+化疗 6 李明等(2007)[9] 女 14 C2-C5 全切 颈部活动受限，左上肢麻木、感觉
异常生殖细胞瘤无STB 放疗 4个月无复发 7 Aoyama等(2007)[10] 女 16 T9-T1 全切 腰痛，步态不稳，双下肢轻瘫，尿潴
留，血β-HCG 0.9 U/L，CSF β-HCG 7.3 U/L生殖细胞瘤无STB 放疗+化疗 45个月无复发 8 林坤花(2007)[11] 男 8 L1-L2、
圆锥、马尾全切 双下肢疼痛，行走不便，尿潴留，血
β-HCG 12 800 U/L生殖细胞瘤含STB 放疗 6年无复发 9 Tubbs等(2008)[12] 男 14 脊髓圆锥 全切 先天性肾上腺增生症，中央性性早
熟，尿失禁生殖细胞瘤 未放化疗 3个月无复发 10 Madhukar等(2013)[3] 女 11 T6-T7 活检 下肢无力，行走困难 生殖细胞瘤 放疗+化疗完全缓解 6个月后颅内转移 11 本例 男 5 L1-L3 部分切除 臀部及下肢疼痛，血AFP 44.8 μg/
L，血β-HCG 155.3 U/L，CSF AFP
287.2 μg/L，CSF β-HCG 2542 U/L生殖细胞瘤+未成熟畸胎瘤 放疗+化疗 6程化疗后20 d复发、转移 β-HCG:β-人绒毛膜促性腺激素；CSF:脑脊液；STB：合体滋养层细胞；AFP:甲胎蛋白 
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