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縱隔雙側交感與副交感神經纖維瘤病-病例報告及文獻回顧

Bilateral Autonomic Neurofibromatosis in the Mediastinum - A Case Report and Review of the Literature

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摘要


什麼是神經纖維瘤病?很難有一準確、深入的全面回答。因為此病雖皆與神經纖維有關,但臨床表現卻極為錯綜復雜,臨床工作中難以掌握。本文報導累及雙側交感與副交感神經的女性縱隔神經纖維瘤病人,是國內首例。手術切除後,自律神經功能可大部份代償。從臨床實用角度出發,我們以為Riccardi將神經纖維瘤分為I型:皮膚斑及皮膚神經纖維瘤;II型皮膚斑及頭顱或脊髓神經纖維瘤樣病變;III型:所有其它神經纖維瘤病。這種分類法最便於臨床掌握使用。

並列摘要


We describe a case of neurofibromatosis involving the mediastinum in a 34-year-old woman. The patient pre-sented with bilateral nervous system involvement, including a sympathetic neurofibroma on the right side and a para-sympathetic neurofibroma on the left side of the mediastinum. Limited dysfunction of the autonomic nervous system persisted even after the tumor was surgically resected. To the best of our knowledge, this is the first case of neurofibromatosis involving bilateral side in the mediasinum to be reported in China. Riccardi’s classification scheme was found to be suitable for clinical application.

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