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題 名 | 皮膚惡性周邊神經髓鞘腫瘤之不同臨床表現=Malignant Peripheral Nerve Sheath Tumor of the Skin in Various Presentation |
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作 者 | 鄭惠文; 楊佳懿; 吳啟欽; 宋奉宜; 張廷安; | 書刊名 | 中華皮膚科醫學雜誌 |
卷 期 | 22:1 2004.03[民93.03] |
頁 次 | 頁79-87 |
分類號 | 415.692 |
關鍵詞 | 皮膚; 惡性周邊神經髓鞘腫瘤; 神經纖維瘤; Malignant peripheral nerve sheath tumor; Skin; Neurofibromatosis type 1; Neurofibroma; Malignant transformation; |
語 文 | 中文(Chinese) |
中文摘要 | 惡性週邊神經髓鞘腫瘤是從神經組織、神經纖維瘤或是呈現神經髓鞘分化的梭狀細胞肉瘤。相較於深部軟組織惡性週邊神經髓鞘腫瘤,皮膚惡性週邊神經髓鞘腫瘤更是少見。顯微鏡下,梭狀細胞腫瘤主要分佈在真皮,細胞皆呈不規則分佈,高細胞密度及細胞多形性;腫瘤細胞有波浪或逗點狀細胞核並排列成渦紋狀或束狀形式,高核染質和非典型有絲分裂非常顯著。免疫組織化學檢查支持腫瘤細胞有神經分化的證據。臨床上,若發現逐漸長大的皮膚腫瘤,病理組織學檢查發現為多形性梭狀腫瘤細胞,則鑑別診斷應該要考慮皮膚惡性週邊神經髓鞘腫瘤。我們報告三個皮膚惡性週邊神經髓鞘腫瘤的病例,臨床為逐漸增大的皮膚腫瘤,並分別表現為單發型、合併神經纖維瘤及第一型神經纖維瘤病。 |
英文摘要 | Malignant peripheral nerve sheath tumor (MPNST) was a spindle cell sarcoma, which could arise from nerve tissue, neurofibroma or showing nerve sheath differentiation. Cutaneous MPNST was especially rare compared with the tumors of deep soft tissue. Microscopically, all tumor cells revealed irregular contours, high cellularity and pleomorphism, located mainly in the dermis. These tumor cells were all with wavy or comma-shaped nuclei arranged in storiform or fascicular pattern. Hyperchromatism and atypical mitosis were quite prominent. Immunohistochemical studies demonstrated the evidence of neural differentiation. Clinically, an insidiously growing skin tumor which showed pleomorphic spindle ferential diagnosis. Herein, we reported 3 cases of cutaneous MPNST with unique presentation of gradually enlarging skin tumors, which were considered of sporadic type, associated with neurofibroma and neurofibromatosis type 1 respectively. |
本系統中英文摘要資訊取自各篇刊載內容。