Tumor induced osteomalacia: associated with elevated circulating levels of fibroblast growth factor-7 in addition to fibroblast growth factor-23
Tumor induced osteomalacia: associated with elevated circulating levels of fibroblast growth factor-7 in addition to fibroblast growth factor-23
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DOI：
10.5414/cn108596
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发表时间：
2016-01-01
影响因子：
1.1
通讯作者：
Fanti, Paolo
中科院分区：
文献类型：
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作者：
Bansal, Shweta;Khazim, Khaled;Fanti, Paolo
肿瘤性骨软化症是一种罕见的副肿瘤综合征，其特征是肾内磷酸盐消耗、低磷血症和骨软化症。成纤维细胞生长因子-23是一种磷酸化激素，即促尿磷激素，常与钛白斑的发病有关。然而，关于与钛相关的肿瘤表达的其他磷酸素的循环水平和临床意义的信息非常有限。此外，对原发肿瘤的识别构成了TIO治疗中经常遇到的主要挑战。在这里，我们报告了一位临床诊断为TIO的患者，其血中磷酸化的成纤维细胞生长因子-23和成纤维细胞生长因子-7水平升高，并对原发肿瘤进行了广泛但没有回报的放射学检查。在选择性静脉采样中，在左股静脉和髂静脉均显示出最高浓度的成纤维细胞生长因子-23和成纤维细胞生长因子-7，尽管成纤维细胞生长因子-7的偏侧化比成纤维细胞生长因子-23更明显。这一实验室发现使我们能够将重点放在左下肢作为原发肿瘤的可能位置。我们的病例首次表明可以在循环中分析成纤维细胞生长因子-7，并将其用于辅助诊断和定位与钛相关的肿瘤。
Tumor induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterized by renal phosphate wasting, hypophosphatemia, and osteomalacia. Fibroblast growth factor (FGF)-23, a phosphatonin i.e., phosphaturia-promoting hormone, is commonly implicated in the pathogenesis of TIO. However, very limited information is available about the circulating levels and clinical significance of other phosphatonins that are expressed by TIO-associated tumors. In addition, identification of the primary tumor constitutes a frequent major challenge in the management of TIO. Here, we report a patient with the clinical diagnosis of TIO with elevated blood levels of the phosphatonins FGF-23 and FGF-7; and extensive but unrewarding radiological search for the primary tumor. In selective venous sampling, both FGF-23 and FGF-7 displayed highest concentrations in the left femoral and iliac veins; although lateralization was much more pronounced for FGF-7 than FGF-23. This laboratory finding allowed us to focus on the left lower extremity as the likely location of the primary tumor. Our case is the first to show that FGF-7 can be analyzed in the circulation and used to assist in the diagnosis and localization of TIO-associated tumors.