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題 名 | Perivascular Epithelioid Cell Tumor (PEComa) in the Ascending Colon: A Case Report=升結腸血管旁類上皮細胞瘤:病例報告 |
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作 者 | 陳建彰; 劉建國; 林秉緯; | 書刊名 | 中華民國大腸直腸外科醫學會雜誌 |
卷 期 | 28:3 2017.09[民106.09] |
頁 次 | 頁159-164 |
分類號 | 416.245 |
關鍵詞 | 血管旁類上皮細胞瘤; 結節硬化症; 升結腸; Perivascular epithelioid cell tumor; PEComa; TSC; |
語 文 | 英文(English) |
中文摘要 | 血管旁類上皮細胞瘤 (perivascular epithelioid cell tumor) 是一群由類上皮細胞所形成的罕見腫瘤。這些類上皮細胞的特徵是會圍繞著血管分布,並可以同時表現出具黑色素細胞分泌功能的標記,以及肌肉組織的標記。此類腫瘤多發生於軟組織,有一些病例被報告發生於腎臟、肝臟、子宮等臟器中,但發生於結腸的病例很少。由於病例稀少,目前尚無標準治療方針與準確地預測病人臨床表現。我們報告一位升結腸血管旁類上皮細胞瘤的病例,表現症狀是右側腹痛及便血。經大腸鏡和電腦斷層掃描發現升結腸腫瘤併腸套疊。病患接受右側結腸切除手術,經過病理切片及免疫染色多種抗體檢查後確診是血管旁類上皮細胞瘤。部分血管旁類上皮細胞瘤和結節硬化症(Tuberous Sclerosis Complex; TSC)有關,部分血管旁類上皮細胞瘤和結節硬化症的基因改變相似。 |
英文摘要 | Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor composed of perivascular epithelioid cell (PEC).1 The PEC is characterized by epithelioid appearance, clear to eosinophilic granular cytoplasm, perivascular location, and coexpression of smooth muscle and melanocytic markers.^(1-5) The PEC-related tumors include angiomyolipoma (AML), clear cell sugar tumor of the lung (CCST), lymphangioleiomyomatosis (LAM), and very rare tumors in other locations. Many PEComas have been associated with the tuberous sclerosis complex, an autosomal dominant disorder characterized by seizures, mental retardation, and the development of tumors in multiple organs including the brain and skin.1,6,7 We report a case of colonic PEComa with personal and family history of tuberous sclerosis complex (TSC). |
本系統中英文摘要資訊取自各篇刊載內容。