摘要
痕跡性腦脊髓膜膨出是一種原發性皮膚腦膜瘤的變異型，是罕見的胚胎學上的畸形。我們研究五個病例，他們自出生即在枕部發現有一塊禿髮的病灶。在腦部電腦斷層或超音波下，沒有發現骨骼缺陷或與顱內相通部份。病理下，主要變化集中在皮下組織，可以看到一縷縷嵌於緻密膠原組織的腦脊髓膜細胞。這些細胞在免疫染色下可表現vimentin及上皮膜抗原(epithelial membrane antigen, EMA)，但缺乏細胞角質，S-100蛋白，平滑肌肌動蛋白及內皮細胞標記。我們提出此罕見疾病，並且回顧相關文獻。
關鍵字
無資料並列摘要
Rudimentary meningocele, a variant of primary cutaneous meningioma, is a rare embryologic malformation with meningeal tissue occurring in the skin. This work studied five cases of this condition. All five cases were congenital and presented as alopecic lesions on the occipital area. No underlying bony defect or communication to the intracranial compartment was detected by brain computed tomography (CT) or echoencephalogram. Histologically, the major changes were mostly concentrated in the subcutis, where strands of meningothelial cells were embedded in dense collagenous tissue. The meningothelial cells were immunopositive for vimentin and epithelial membrane antigen and lacked cytokeratin, S-l00 protein, smooth muscle actin and endothelial markers. The related literature also was reviewed.
延伸閱讀
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