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題 名 | Familial Neurofibromatosis with Mediastinal Cystic Neurofibroma=家族性神經纖維瘤合併囊狀縱膈神經纖維瘤 |
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作 者 | 陳威男; 蔡聰聰; 陳重華; | 書刊名 | 胸腔醫學 |
卷 期 | 14:2 1999.06[民88.06] |
頁 次 | 頁62-66 |
分類號 | 415.452 |
關鍵詞 | 多發性神經纖維瘤; 神經纖維瘤; 神經瘤; 縱膈腔腫瘤; Mediastinal tumor neurofibroma; Neurofibromatosis; Neurogenic tumor; |
語 文 | 英文(English) |
中文摘要 | 神經纖維瘤是一種顯性遺傳疾病,常以皮膚上的咖啡牛奶色斑及神經 纖維瘤表現。肺部侵犯並不常見,可有間質性纖維化,大型氣泡,且常合併神經 瘤。我們提出一例以縱膈腔囊狀神經纖維瘤為表現的中年女性。其家族成員有 39%(5/13)表現出咖啡牛奶色斑皮膚神經纖維瘤,病患僅偶爾有背痛及下肢無力 情形,胸部X光顯示出一個界限完整的縱膈腔腫瘤,磁振造影顯現其為一啞鈴 型硬膜內及髓質外的腫瘤且併有神經開口擴大。病患接外科手術切除,病理報告 為囊狀神經纖維瘤。我們將對神經纖維瘤的臨床及影像表現做一探討。 |
英文摘要 | Neurofibromatosis, manifesting as cutaneous cafe au lait spots and neurofibromas of the skin, is an autosomal dominant heritable disease. Infrequent pulmonary manifestations which include interstitial fibrosis and bullae formation are often combined with an intrathoracicneurogenic tumor. A case of cutaneous neurofibromatosis with mediastinal cystic neurofibromas is reported. Cutaneous cafe au lait spots and neurofibromas of the skin were noted among 39%(5/13) of her family. The patient complained of occasional lumbago and lower extremity weakness. Chest roentgenogram showed a well-defined posterior mediastinal tumor and MRI of T-spine showed an intradurally and extradurally located dumbell-shaped mass with widening of its neural foramina. Surgical excision was done and cystic neurofibroma was confirmed by pathology. The clinical manifestations and roentgenographic findings of neurofibromatosis are reviewed. |
本系統中英文摘要資訊取自各篇刊載內容。