本文選題：腫瘤性骨軟化癥　切入點：成纖維細胞生長因子　出處：《中華骨質疏松和骨礦鹽疾病雜志》2016年02期 　論文類型：期刊論文

【摘要】：腫瘤性骨軟化癥(tumor induced osteomalcia,TIO)是一種代謝性骨病,是由于腫瘤分泌成纖維細胞生長因子23(FGF-23),導致腎磷排出增加、骨礦化障礙,以進行性骨痛、乏力、骨軟化為臨床表現。TIO腫瘤主要來自于骨組織及軟組織,被稱之為磷酸鹽尿性間葉組織腫瘤(phosphaturic mesenchymal tumor,PMT)。主要病理表現為鏡下可見的大量梭形細胞,間葉組織來源,血管豐富,可見破骨樣細胞、骨及軟骨樣結構,可見鈣化,免疫組化染色表達FGF-23。其特點在于豐富的血管,尤其是畸形厚壁血管。目前,對于PMT發生,產生FGF-23的機制不明。腫瘤發生、發展具有多種因素影響,一方面,目前研究認為內皮細胞-間充質細胞轉化(endothelial-to-mesenchymal transformation,End MT)是腫瘤發展,具有侵襲性的可能機制之一,參與多種腫瘤的發展過程。另一方面,FGFR的過度激活與腫瘤的發生相關,FGFR的融合基因可與乳腺癌,前列腺癌等相關。FGFR與骨腫瘤的研究很少,但有研究表明FGFR1與纖維連接蛋白1(fibronectin1,FN1)的融合基因在PMT腫瘤中多見。另外,FGFR1激活后可促進FGF-23表達。這些機制可能與TIO腫瘤有關。
[Abstract]:Tumor induced osteomalacia (tumor induced, osteomalcia, TIO) is a metabolic bone disease, is due to the tumor secretion of fibroblast growth factor 23 (FGF-23), leading to renal phosphate excretion, impaired bone mineralization, to pain, fatigue, bone softening for the clinical manifestations of.TIO tumor mainly from bone tissue and soft the organization, known as the phosphate urinary mesenchymal tumor (phosphaturic mesenchymal, tumor, PMT). The main pathological features visible under the microscope for a large number of spindle shaped cells, vascular mesenchymal tissue source rich, visible osteoclast like cells, bone and cartilage like structure, calcification, immunohistochemical staining, the expression of FGF-23. is rich in blood vessels, especially abnormal thick walled vessels. At present, the incidence of PMT, mechanisms of FGF-23 are unknown. The tumor development, with a variety of factors, on the one hand, current studies suggest that endothelial cells - mesenchymal transition (e Ndothelial-to-mesenchymal transformation, End MT) is one of the possible mechanisms of tumor development, aggressive development process, participate in a variety of tumors. On the other hand, the excessive activation of FGFR and tumor associated FGFR fusion gene and breast cancer, prostate cancer and other related research rarely.FGFR and bone tumor, but studies have shown that the FGFR1 and fibronectin 1 (fibronectin1, FN1) fusion gene in PMT tumor in common. In addition, the activation of FGFR1 can promote the expression of FGF-23. The mechanism may be related to TIO tumor.

【作者單位】： 中國醫學科學院北京協和醫學院北京協和醫院內分泌科國家衛生和計劃生育委員會(衛生部)內分泌重點實驗室;
【基金】：國家自然科學基金面上項目(81070687,81170805)
【分類號】：R681

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