Division of Molecular Pathology, Department of Microbiology and Pathology, Tottori University Faculty of Medicine, Yonago, Japan.
Neuropathology. 2011 Dec;31(6):632-8. doi: 10.1111/j.1440-1789.2011.01209.x. Epub 2011 Mar 29.
Spinal cord tumors are rare in children. We report a novel case of pediatric intramedullary spinal cord tumor with unusual solid-cystic and papillary features. Clinically, the patient presented at the age of 3 years with motor deficit and urinary incontinence, and MRI demonstrated multilocular cystic lesions in the thoracic spine. Histologically the tumor consisted of solid, sheet-like components and branching papillary structures, and immunohistochemistry demonstrated positive reactivity for epithelial membrane antigen, cytokeratins (7, AE1/3, CAM5.2), E-cadherin and transthyretin, and negativity for GFAP, S-100 protein, synaptophysin and neurofilament. These histological and immunohistochemical findings appeared to be unique, and were not compatible with the features of classical ependymoma or choroid plexus papilloma. The clinical behavior, characterized by relatively rapid tumor regrowth after surgical resection and a relatively high MIB-1 labeling index, suggest that this tumor might have had moderate malignant potential. This pediatric case appears to be particularly informative with regard to the tumor biology or tumorigenesis of intramedullary spinal cord tumor with unusual solid-cystic and papillary features.
脊髓肿瘤在儿童中较为罕见。我们报告了一例具有独特实性-囊性和乳头状特征的小儿脊髓髓内肿瘤的新病例。临床方面，患者 3 岁时表现为运动功能障碍和尿失禁，MRI 显示胸椎多房囊性病变。组织学上，肿瘤由实性、片状成分和分支状乳头状结构组成，免疫组化显示上皮膜抗原、细胞角蛋白（7、AE1/3、CAM5.2）、E-钙黏蛋白和转甲状腺素阳性反应，而神经胶质纤维酸性蛋白、S-100 蛋白、突触素和神经丝呈阴性。这些组织学和免疫组化表现似乎是独特的，与经典室管膜瘤或脉络丛乳头状瘤的特征不符。临床行为表现为手术切除后肿瘤较快复发，且 MIB-1 标记指数相对较高，提示该肿瘤可能具有中等恶性潜能。这个小儿病例在具有独特实性-囊性和乳头状特征的脊髓髓内肿瘤的肿瘤生物学或肿瘤发生方面似乎特别有意义。
