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Clinical Characteristics of Malignant Phosphaturic Mesenchymal Tumor causing Tumor Induced Osteomalacia.
The Journal of Clinical Endocrinology & Metabolism ( IF 5.8 ) Pub Date : 2023-11-25 , DOI: 10.1210/clinem/dgad690
Veronica Abate 1 , Anita Vergatti 1 , Gianpaolo De Filippo 2 , Vincenzo Damiano 3 , Ciro Menale 1 , Lanfranco D'Elia 1 , Domenico Rendina 1
Affiliation  

PURPOSE Tumor induced osteomalacia (TIO) is a rare paraneoplastic syndrome usually caused by over-secretion of Fibroblast Growth Factors 23 (FGF23) from phosphaturic mesenchimal tumour (PMT). PMT are usually benign neoplasms but some of them show malignant characteristics. Aim of this study was to compare the clinical characteristics of benign and malignant PMT inducing TIO. METHODS On 31th March 2023, we performed a systematic review with individual patients data analysis in Medline, Google Scholar, Google book, and Cochrane Library using the terms: "tumor induced osteomalacia, " "oncogenic osteomalacia", "hypophosphatemia", with no language restrictions and according to Preferred Reporting Items for Systematic reviews and Meta-Analyses criteria. RESULTS Overall, we collected data from 837 TIO patients in which the diagnosis of benign and malignant PMT was specified. Of them, 89 were affected by malignant PMT and 748 by benign PMT. Patients with malignant PMT were younger and presented more frequently bone pain, functional impairment, and bone deformities. The malignant PMT showed higher values of intact FGF23 and a higher mortality rate. CONCLUSION The study results identify the clinical characteristics of patients with malignant TIO, permitting the early identification of PMT patients at increased risk of malignancy. This may significantly improve the diagnostic approach to disease. Further experimental studies are mandatory to clarify the FGF23 role in the pathogenesis of malignancy in PMT.

中文翻译:

导致肿瘤诱发骨软化的恶性磷酸盐间叶瘤的临床特征。

目的肿瘤诱发的骨软化症 (TIO) 是一种罕见的副肿瘤综合征,通常由磷酸盐性间质肿瘤 (PMT) 过度分泌成纤维细胞生长因子 23 (FGF23) 引起。PMT 通常是良性肿瘤,但其中一些表现出恶性特征。本研究的目的是比较良性和恶性 PMT 诱导 TIO 的临床特征。方法 2023 年 3 月 31 日,我们使用以下术语对 Medline、Google Scholar、Google 图书和 Cochrane 图书馆中的个体患者数据分析进行了系统回顾:“肿瘤诱发的骨软化症”、“致癌性骨软化症”、“低磷血症”,但没有任何语言限制并根据系统评价和荟萃分析标准的首选报告项目。结果 总体而言,我们收集了 837 名 TIO 患者的数据,其中明确了良性和恶性 PMT 的诊断。其中,89 例受恶性 PMT 影响,748 例受良性 PMT 影响。恶性 PMT 患者更年轻,更容易出现骨痛、功能障碍和骨畸形。恶性PMT表现出较高的完整FGF23值和较高的死亡率。结论 研究结果确定了恶性 TIO 患者的临床特征,有助于及早识别恶性肿瘤风险增加的 PMT 患者。这可能会显着改善疾病的诊断方法。必须进行进一步的实验研究来阐明 FGF23 在 PMT 恶性肿瘤发病机制中的作用。
更新日期:2023-11-25
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