椎管内节细胞神经瘤12例临床病理学分析

目的:探讨椎管内节细胞神经瘤的临床与病理特征.方法:收集12例椎管内节细胞神经瘤的病例,以1例发生于纵隔的节细胞神经瘤为对照,进行临床、症状、体征、影像学特点分析;标本经常规检查及NF,S-100,MBP,PMP22,Ki-67,SMA,GFAP免疫组织化学染色,对其病理学和临床特点进行分析.结果:12例椎管内节细胞神经瘤发生于30～40岁.女性较多发.部位均位于颈髓节段,临床上多以根性疼痛及脊髓压迫症状为主.形态学上可见成熟或未成熟的肿瘤性节细胞散在或成束穿插于肿瘤性Schwann细胞间质中,肿瘤中可见正常的神经纤维呈束状排列.免疫组化:NF(节细胞+),S-100(含神经鞘瘤样结构的肿瘤成分+,高于含神经纤维瘤样结构者;另外节细胞周围的卫星细胞+).MBP(成熟的神经纤维+),PMP22(弥漫+),Ki-67(含神经鞘瘤结构的区域约3%,其余区域0～1%).SMA(血管内皮无明显增生).GFAP结果不恒定.结论:发生在椎管内的节细胞神经瘤较罕见,肿瘤本身为良性(WHO I级).常引起根性疼痛.由于发生部位为椎管内甚至伴行神经根长出椎间孔,因此在形态学上要与发生在椎间孔区的粘连了神经根的神经鞘瘤或神经纤维瘤相鉴别.手术切除的完全与否对预后影响大.

Clinicopathologic Analysis of 12 Cases of Intraspinal Ganglioneuroma

Objective:To investigate the clinicopathologic features of intraspinal ganglioneuroma.Meth-ods:We collected 1 2 cases of diagnosed ganlioneuroma arising from the spine and one case of ganlioneuro-ma arising from mediastinum as the control.Clinical and radiographic features were reviewed.The pathologi-cal parameters of these cases were analyzed with routine and immunohistochemical stainings of neural fiber (NF),S-100 protein(S-100),myelin based protein(MBP),peripheral myelin protein 22(PMP22),smooth mus-cle actin(SMA),glial fibrilary acidic protein(GFAP)and Ki-67.Results:The disease was likely to occur in pa-tients aged 30-40 years old and more common in female.These cases were all intral cervical spinal tumors and presented with radicular neuralgia and mass effects of cervical spinal cord compression.Ganglioneuro-mas which occasionally contained normal spindle shaped cells were composed of mature or degenarative ganglion cells and neoplastic Schwannian stroma.Ganglion cells appeared positive for NF.Schwannian stro-ma as well as satellite cells around ganglion showed immunoreactivity for S-100.more intense than neurofi-bro-stroma.Mature spindle shaped cells showed immumoreactivity for MBP.Ki-67 labeling indices were usual-ly 0-1%while in Schwannian stroma areas were 3%.No blood vessel endothelium proliferation was ob-served.Conclusion:Intraspinal ganglioneuromas are rare benign tumors(WHO grand I),causing radicular neuralgia.It is jmportant to distinguish ganglioneuroma with spinal root encircled from Schwannoma or neuro-fibroma in the same anatomic location.The optimal treatment is surgical total resection.