Rumana M, Santosh Vani, Khursheed N, Yasha T C, Kolluri V R S, Shetty S, Ravi Kumar C V
Department of Neuropathology, NIMHANS, Bangalore.
Indian J Pathol Microbiol. 2007 Jul;50(3):528-32.
Spinal paragangliomas are uncommon neoplasms and subject of much debate regarding the factors governing their biological behaviour. We describe the clinicopathological and immunohistochemical (IHC) features of six cases of spinal paraganglioma. The mean age of patients was 40 years (range 20-60 years) with a male to female ratio of (2:1). Majority presented with low backache, sphincter disturbances and sensory symptoms. All tumors were intradural in the cauda region one of them extending to the filum. Only one showed focal extradural extension on microscopy. Gross total resection of tumors was possible in all cases. Histologically four showed classical 'zell-ballen' pattern and two revealed an ependymal morphology. On immunohistochemistry, in all the six cases the chief cells were intensely labeled by antibody to chromogranin but not for GFAP while staining for synaptophysin was less intense and variable in five. Sustentacular cells in all cases showed strong expression for S-100 protein and chief cells were stained light in three cases. Low MIB-1 labeling index of 0.01-2% was noted in five cases and in the sixth it was 5%. None of the tumours recurred. Immunohistochemistry assisted in differentiating these relatively benign neural crest tumours from the more aggressive spinal ependymomas.
脊髓副神经节瘤是罕见的肿瘤，关于其生物学行为的影响因素存在诸多争议。我们描述了6例脊髓副神经节瘤的临床病理及免疫组化（IHC）特征。患者的平均年龄为40岁（范围20 - 60岁），男女比例为2:1。多数患者表现为腰痛、括约肌功能障碍及感觉症状。所有肿瘤均位于马尾区硬膜内，其中1例延伸至终丝。仅1例在显微镜下显示局灶性硬膜外扩展。所有病例均可行肿瘤全切除。组织学上，4例显示典型的“细胞巢”模式，2例呈现室管膜形态。免疫组化方面，6例中所有主细胞均被嗜铬粒蛋白抗体强烈标记，但对胶质纤维酸性蛋白（GFAP）无反应，而突触素染色在5例中较弱且不一致。所有病例中的支持细胞均对S - 100蛋白呈强表达，3例主细胞呈淡染色。5例的MIB - 1标记指数较低，为0.01% - 2%，第6例为5%。所有肿瘤均未复发。免疫组化有助于将这些相对良性的神经嵴肿瘤与侵袭性更强的脊髓室管膜瘤区分开来。
