脊髓脊膜膨出14例病理诊断及鉴别诊断分析

Pathological diagnosis and differential diagnosis of myelomeningoceles:an analysis of 14 cases

目的探讨脊髓脊膜膨出的病理形态学特征、病理诊断标准和鉴别诊断。方法回顾分析14例脊髓脊膜膨出患者的临床资料、组织学特征并行免疫组化研究。结果患儿年龄1天～4岁,平均11.4个月,男女各7例。病变发生在胸、腰椎部位的2例、骶尾部12例。病理形态特征包括:病变中央部位的皮肤附件数量减少或缺如,真皮层内可见多量血管增生,病灶边界不清;14例均可见神经胶质组织、脊膜组织与胶原纤维混合形成的裂隙样结构,局部可见丰富的小神经束和平滑肌束;6例病变可见奇异形细胞和多核细胞散在分布。免疫组化:神经胶质组织GFAP(+);裂隙样结构内衬的细胞EMA、vimentin、D2-40和SMA(+),而CD31(-);奇异形细胞和多核细胞vimentin和SMA(+)。结论神经胶质组织和内衬脊膜细胞的裂隙样结构是脊髓脊膜膨出病理诊断依据,病灶中平滑肌束的出现对诊断有提示意义。奇异形细胞和多核细胞可能是退变的肌纤维母细胞。熟悉脊髓脊膜膨出的形态特征对病理诊断和鉴别诊断十分重要。

Objective To investigate the pathological morphology criteria for the diagnosis and differential diagnosis of myelomeningoceles. Methods Clinical data from 14 patients with myelomeningoceles were analyzed, retrospectively. The morphological features and immunophenotypes were observed by light microscopy and immunohistochemistry. Results The patients＇ age ranged from 1 day to 4 years with equal sex distribution. The pathological characteristics were diminished or absent dermal appendages of the skin in the central area overlying the lesion, and increased blood vessels in the dermis. The glial components and the plexus of sinusoidal spaces, formed by the spinal meninges and the collagen, were observed in all 14 cases of the poorly circumscribed lesions. Bizarre cells and multinucleated cells were observed in six cases. The cells lining the sinusoidal spaces were positive for EMA, vimentin, D240, SMA, and negative for CD31. Bizarre cells and multinucleated cells were positive for vimentin and SMA. Conclusion Plexus of sinusoidal space, with the lining cells demonstrating spinal meningeal features, as well as the glial components are the hallmark structures for the diagnosis of myelomeningoceles. Bizarre cells and muhinucleated cells are probably degenerative myofibroblasts in nature. It is important to be familiar with the morphological features of myelomeningoceles for the pathological diagnosis and differential diagnosis.