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皮膚多發性叢狀神經鞘瘤─病例報告及文獻回顧─

Multiple Cutaneous Plexiform Schwannomas -A Case Report and Review of the Literature-

摘要


叢狀神經鞘瘤(plexiform schwannoma)是良性神經鞘瘤的罕見變異型。相對於叢狀神經纖維瘤(plexiform neurofibroma),叢狀神經鞘瘤與第一型予纖維瘤病(neurofibromatosis type 1,von Recklinghausen’s disease)的相關很薄弱,而且沒有惡性變化的傾向。叢狀神經鞘瘤大部分 都是單獨的病灶,只有少數的病例是多發的,而這些多發性叢狀神經鞘瘤可能合併中樞神經系統的腫瘤而與第二型神經纖維瘤病有重疊的臨床表現。我們報告一例局限於皮膚的多發性叢狀神經鞘瘤,並回顧文獻中所提及與第二型神經纖維瘤病的關係。

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並列摘要


Plexiform schwannoma is a rare variant of the typical schwannoma. In contrast to plexiform neurofibromas, plexiform schwannoma is only weakly associated with neurofibromatosis type 1 (von Recklinghausen’s disease), and has little malignant potential. Most plexiform schwannomas are solitary, and only a minority of reported cases are multiple. The latter may be associated with central nervous system tumors and show overlapping clinical features with neurofibromatosis type 2. We herein report a case of multiple plexiform schwannomas limited to the skin, and review the literature regarding the association with neurofibromatosis type 2.

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