丛状神经纤维瘤12例临床病理分析

    Plexiform neurofibroma: Clinicopathological analyses of 12 cases

    • 摘要: 目的: 探讨丛状神经纤维瘤的临床病理学特征。方法: 对12例丛状神经纤维瘤的临床表现、病理学特征进行观察和分析,并结合文献进行讨论。结果: 丛状神经纤维瘤一般在儿童期发病,好发头颈部、四肢和躯干。组织学表现为由Schwann细胞、纤维母细胞及胶原纤维构成的丛状结构。免疫组化标记显示:梭形细胞以S-100蛋白阳性为主,vimentin、EMA、NSE或NF散在阳性。结论: 丛状神经纤维瘤有特殊的临床病理学特征,提高对丛状神经纤维瘤的认识有助于排查神经纤维瘤病1型。

       

      Abstract: Objective: To explore the clinicopathological features of plexiform neurofiroma(PNF).Methods: The clinical manifestatous and pathological charactors of 12 cases of PNF were observed and analysed combined with review of the literature.Results: PNF developed in childhood generally and often occurred at head and neck,limbs and trunks.The tumor appeared plexiform pattern consisted of Schwann cells,fibroblasts and collagen fibers.The spindle cells expressed S-100 protein diffusely and vimentin,EMA,NSE and NF scattered.Conclusions: PNF is a tumor with special clinicopathological characteristics,which is helpful to recognize the neurofibromatosis 1(NF1).

       

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