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題名 | Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report=伴隨血清中CA 125升高的腹腔內促纖維化小圓細胞腫瘤--病例報告 |
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作者 | 楊曉芳; 王勝嵐; 蔡志仁; 蘇裕傑; 歐陽賦; 陳巧雲; Yang, Sheau-fang; Wang, Sheng-lan; Chai, Chee-yin; Su, Yu-chieh; Ou-yang, Fu; Chen, Chau-yun; |
期刊 | The Kaohsiung Journal of Medical Sciences |
出版日期 | 20031000 |
卷期 | 19:10 2003.10[民92.10] |
頁次 | 頁531-536 |
分類號 | 415.57 |
語文 | eng |
關鍵詞 | 促纖維化小圓細胞腫瘤; 腹腔內; Desmoplastic small round cell tumor; Intra-abdominal; CA 125; |
中文摘要 | 促纖維化小圓細胞腫瘤是一種罕見的高度惡性腫瘤,通常發生於腹膜,特別容易侵犯年輕男性。它在臨床表現、組織學和免疫組織學上都有典型的特徵。本病組織形成的機轉尚未被完全了解,然而從它在免疫組織化學染色上同時表現出上皮性、間質性及神經性的抗原來看,可以推測出此腫瘤可能起源於原始有多種作用的幹細胞,並且往多個方向分化而形成。近年來許多細胞遺傳學上的研究認為此腫瘤之所以會往多個方向分化乃是因為腫瘤細胞發生了染色體移位,導致其上的 Ewing 氏肉瘤基因及 Wilms 氏腫瘤抑制基因融合所造成。在臨床上,曾有人報告過病人有血清中 CA 125 增高的情形。本篇報告了一個發生在 29 歲男性腹腔內的促纖維化小圓細胞腫瘤,同時伴隨血清中高濃度的 CA 125。我們回顧相關文獻並討論 CA 125 在本病的代表意義及疾病的治療。 |
英文摘要 | Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive tumor usually involving the peritoneum. It occurs more commonly in young males and is characterized by distinctive clinical, histologic, and immunophenotypic features. The histogenesis of DSRCT remains unknown. Coexpression of epithelial, mesenchymal, and neural antigens in the same cell provides evidence that DSRCT may arise from a primitive pluripotent stem cell with divergent differentiation. Recently, according to cytogenetic studies, some authors have proposed that the divergent differentiation of DSRCT may be the result of the fusion of Ewing’s sarcoma gene and Wilms’ tumor suppressor gene. Clinically, an elevated serum CA 125 concentration is found in some patients with DSRCT. We present the case of a 29-year-old man with diffuse intra-abdominal DSRCT and elevated serum CA 125 concentration and briefly review the relevant literature. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。