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題 名 | Reconstruction Using a Free Lateral Leg Perforator Flap after Excision of a Malignant Peripheral Nerve Sheath Tumor of the Hand--A Case Report and Literature Review=手部惡性周邊神經鞘膜瘤術後以小腿外側穿通枝皮瓣重建--病例報告及文獻探討 |
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作 者 | 李柏穎; 黃世鴻; 顏毓秀; 蒲啟明; | 書刊名 | 臺灣整形外科醫學會雜誌 |
卷 期 | 25:3 2016.09[民105.09] |
頁 次 | 頁258-268 |
分類號 | 416.615 |
關鍵詞 | 手部; 惡性周邊神經鞘膜瘤; 小腿外側穿通枝皮瓣; Malignant peripheral nerve sheath tumor; Moft tissue sarcoma; Hand; Lateral leg perforator flap; |
語 文 | 英文(English) |
中文摘要 | 背景:惡性周邊神經鞘膜瘤是一種具有高復發率、低存活率、且相當罕見的惡性肉瘤。原發性的手部惡性周邊神經鞘膜瘤更是極為少見,文獻上僅有少數的病例報告。目的及目標:報告一位手部惡性周邊神經鞘膜瘤的病例,作經驗分享及相關文獻探討。材料及方法:一位六十二歲男性,本身無第一型神經纖維瘤的特徵,也無家族病史,以左手食指壺口部一個無痛性且漸長大的腫塊為臨床表現。在第一次腫瘤切除時,發現腫瘤與食指的橈掌側固有神經沾黏在一起。術後病理組織分析為惡性周邊神經鞘膜瘤,且組織邊緣仍有腫瘤細胞浸潤。影像檢查並無遠端轉移情形。一個月後,病人接受廣泛性腫瘤切除以及食指和中指截肢術,達成了邊緣無腫瘤細胞浸潤,並以小腿外側穿通枝皮瓣來重建手部缺損。結果:皮瓣成功存活且無任何併發症,病人術後追蹤兩年無腫瘤復發之情形,其手部功能維持良好,對於重建後外觀亦感到滿意。結論:外科手術是惡性周邊神經鞘膜瘤的的主要治療方式。對於手部惡性周邊神經鞘膜瘤,要避免有不完整的腫瘤切除情形,也要避免有過多正常組織切除情形。在達到廣泛性腫瘤切除且邊緣無腫瘤浸潤的目標下,要盡量行手部功能保留手術。 |
英文摘要 | Background: Malignant peripheral nerve sheath tumor (MPNST) is a rare form of soft tissue sarcoma with high recurrence rates and poor survival outcomes. Primary MPNST arising from the hand is extremely rare. Only isolated case reports regarding hand MPNSTs have appeared in the literature. Aim and Objectives: In this report we present our experience managing a patient with MPNST of the hand, and review the relevant literature. Materials and Methods: A 62-year-old man with no clinical signs or family history of neurofibromatosis type 1 presented with an asymptomatic soft tissue mass increasing in size over the first volar web of his left index finger. The tumor was attached to the radial proper palmar digital nerve of the index finger during initial en bloc excision. The histopathologic analysis revealed MPNST with a positive margin. The imaging studies showed no distant metastasis. One month later, the patient underwent wide amputation of the index and long fingers that achieved a negative margin status. A free proximal lateral leg perforator flap was used for defect reconstruction. Results: The flap survived completely, without any sequelae. The patient was disease free at nearly 2 years of follow-up. Excellent hand function was retained, and the esthetic result of the reconstructed hand was satisfactory. Conclusions: The nature of MPNST is highly aggressive. To date, surgical excision with tumor -free margins remains the mainstay of treatment. For hand MPNSTs, the surgeon performing oncologic resection should avoid either incomplete or unnecessary resection. When feasible, wide-margin and function-sparing surgical excision is the cornerstone of effective treatment for hand MPNSTs. |
本系統中英文摘要資訊取自各篇刊載內容。