肿瘤性骨软化症病理特点及致瘤基因

发布时间：2018-03-03 07:21

本文选题：肿瘤性骨软化症　切入点：成纤维细胞生长因子　出处：《中华骨质疏松和骨矿盐疾病杂志》2016年02期 　论文类型：期刊论文

【摘要】：肿瘤性骨软化症(tumor induced osteomalcia,TIO)是一种代谢性骨病,是由于肿瘤分泌成纤维细胞生长因子23(FGF-23),导致肾磷排出增加、骨矿化障碍,以进行性骨痛、乏力、骨软化为临床表现。TIO肿瘤主要来自于骨组织及软组织,被称之为磷酸盐尿性间叶组织肿瘤(phosphaturic mesenchymal tumor,PMT)。主要病理表现为镜下可见的大量梭形细胞,间叶组织来源,血管丰富,可见破骨样细胞、骨及软骨样结构,可见钙化,免疫组化染色表达FGF-23。其特点在于丰富的血管,尤其是畸形厚壁血管。目前,对于PMT发生,产生FGF-23的机制不明。肿瘤发生、发展具有多种因素影响,一方面,目前研究认为内皮细胞-间充质细胞转化(endothelial-to-mesenchymal transformation,End MT)是肿瘤发展,具有侵袭性的可能机制之一,参与多种肿瘤的发展过程。另一方面,FGFR的过度激活与肿瘤的发生相关,FGFR的融合基因可与乳腺癌,前列腺癌等相关。FGFR与骨肿瘤的研究很少,但有研究表明FGFR1与纤维连接蛋白1(fibronectin1,FN1)的融合基因在PMT肿瘤中多见。另外,FGFR1激活后可促进FGF-23表达。这些机制可能与TIO肿瘤有关。
[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.

【作者单位】：中国医学科学院北京协和医学院北京协和医院内分泌科国家卫生和计划生育委员会(卫生部)内分泌重点实验室;
【基金】：国家自然科学基金面上项目(81070687,81170805)
【分类号】：R681

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