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題 名 | Perivascular Epithelioid Cell Tumors (PEComas) Refractory to mTOR Inhibitors=以mTOR抑製劑治療無效的血管周圍上皮樣細胞腫瘤 |
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作 者 | 陳三奇; 楊茜文; 顏厥全; 曾成槐; 趙大中; | 書刊名 | 臺灣癌症醫學雜誌 |
卷 期 | 1:2 2014.09[民103.09] |
頁 次 | 頁128-133 |
分類號 | 415.641 |
關鍵詞 | 血管周圍上皮樣細胞腫瘤; mTOR抑制劑; 特癌適; 癌伏妥; Perivascular epithelioid cell tumors; PEComas; mTOR inhibitors; Temsirolimus; Everolimus; |
語 文 | 英文(English) |
中文摘要 | 血管周圍上皮樣細胞腫瘤是由血管周圍上皮樣細胞所構成的間質腫瘤的統稱。這類 腫瘤非常少見,且有不同的惡性度。TSC1/2 基因的突變可以發生在與結節性硬化症相關 的或偶發性的血管周圍上皮樣細胞腫瘤。在這些腫瘤也發現有 mTORC1 路徑的活化,使 得 mTOR 抑制劑很合理的被嘗試在治療這類的腫瘤。一些報告顯示 mTOR 抑制劑對腫瘤 治療有持久的反應。我們在此報告一位 71 歲患有血管周圍上皮樣細胞腫瘤的女性,復發 後對於特癌適以及癌伏妥的治療無效。 |
英文摘要 | Perivascular epithelioid cell tumors (PEComas) are mesenchymal tumors with a particular perivascular epithelioid cell differentiation. These tumors are extremely rare and represent a form of malignancy with certain characterizations. The TSC1/2 gene mutation can develop in both tuberous sclerosis complex (TSC)-related PEComa and sporadic cases. The mTORC1 pathway activation is also found in these tumors. Currently, mTOR inhibitors have been used for the treatment of PEComas, and some reports have shown durable responses with the use of such mTOR inhibitors. We present a 71-year-old woman who had recurrent PEComa which was refractory to temsirolimus and everolimus. |
本系統中英文摘要資訊取自各篇刊載內容。