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題名 | Mesenteric and Retroperitoneal Malignant Peripheral Nerve Sheath Tumors in a Patient with Neurofibromatosis Type 1=腸繫膜與後腹腔的惡性周圍神經鞘瘤合併第一型多發性神經纖維瘤症 |
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作者姓名(中文) | 吳金珠; 彭惠玲; 蘇誠道; 盧大年; 李進成; 陳良光; | 書刊名 | 中華放射線醫學雜誌 |
卷期 | 28:3 2003.06[民92.06] |
頁次 | 頁193-198 |
分類號 | 415.9413 |
關鍵詞 | 電腦斷層攝影; 惡性周圍神經鞘瘤; 第一型多發性神經纖維瘤; 周圍神經鞘腫瘤; Computed tomography; Malignant peripheral nerve sheath tumor; Neurofibroma; Neurofibromatosis; |
語文 | 英文(English) |
中文摘要 | 惡性周圍神經鞘瘤是一種罕見的高度惡性且預後極差的梭狀細胞性軟組織肉瘤。一般相信它是由“以前存在的”周圍神經鞘腫瘤演化形成或經由放射治療後所產生。它可發生在身體任何部位。將近有50-60%的病人會合併第一型多發性神經纖維瘤症。本文報告一多發性病例,病灶主要位於後腹腔及腸繫膜的惡性周圍神經鞘腫瘤。病人為56歲男性,臨床上沒有特殊的症狀。影像上也沒有較特殊的表徵。雖然最後的診斷需依賴病理切片,但臨床及影像學表現仍扮演著重要角色。此病患除了接受手術切除外,並接受了放射線及化學治療。但因病情惡化及多處轉移而於術後24個月死亡。此文的目的,是想藉以此病例的臨床,影像及組織學表現與治療,來提升放射科醫師對此少見腫瘤之經驗;並針對此做了回溯性探討。 |
英文摘要 | Malignant peripheral nerve sheath tumor (MPNST), an aggressive spindle cell sarcoma is found predominantly in adults. The tumor is closely associated with neurofibromas, neurofibromatosis type 1 (NF-1) and from the sites previously irradi-ated. Individuals with NF-1 account for 50-60% of all of the patients with MPNSTs [1]. Herein, we describes a fatal case of MPNST in NF-1 with atyp-ical age of onset and multicentric tumors at different sites occurred over a short period of time after the initial surgery. The lesion was studied with ultra-sound, computed tomography (CT) and magnetic resonance imaging (MRI). The most characteristic findings in our case was heterogeneously low density masses without any apparent organ-based primary site within the mesentery and retroperitoneum. The diagnosis was established by histologic picture, com-bined with clinical manifestation and imaging find-ings. The characteristic clinical, radiologic and histopathologic features of this process are reviewed along with the diagnostic and therapeutic options. |
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