Reports of 17 Chinese patients with tumor-induced osteomalacia
Reports of 17 Chinese patients with tumor-induced osteomalacia
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DOI：
10.1007/s00774-016-0756-9
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发表时间：
2017-05-01
影响因子：
3.3
通讯作者：
Zhang, Zhen-Lin
中科院分区：
文献类型：
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作者：
Yu, Wei-Jia;He, Jin-Wei;Zhang, Zhen-Lin
肿瘤诱发的骨软化症 (TIO) 是一种罕见的获得性低磷血症性骨软化症，通常归因于良性间叶肿瘤过度产生成纤维细胞生长因子 23 (FGF-23)。肿瘤的定位和随后的手术切除可以治愈。本研究旨在调查上海某医疗中心的临床数据、诊断方法和肿瘤切除后的随访，以描述这种罕见疾病的概况，并分享我们在诊断和治疗方面的成功经验。纳入2009年至2014年上海市第六人民医院收治的23名成人发病的低磷血症骨软化症患者和95名正常人。在询问病史并进行体检后，我们分析了实验室结果（包括血清 FGF-23 水平），并通过 18F-氟脱氧葡萄糖正电子发射断层扫描和计算机断层扫描（18F-FDG PET/CT）、（99）mT-科曲肽（（99）mT-c-OCT）闪烁扫描和磁共振成像（MRI）定位肿瘤。根据实验室检查和影像学检查结果，对17例确诊为TIO的患者进行了肿瘤切除术。结果表明，17 名患者（9 名男性和 8 名女性，平均年龄 46.6 +/- 12.9 岁）患有 TIO。 94.1% 的患者（17 名患者中的 16 名）FGF-23 水平升高。 100% 的患者血清磷水平下降。 F-18-FDG PET/CT 显示 5 个肿瘤，(99)mT-cOCT 闪烁扫描显示 2 个肿瘤，体检显示 9 个肿瘤，MRI 显示 1 个肿瘤，其中 58.8% 的致病肿瘤（17 个肿瘤中的 10 个）位于下肢。肿瘤切除后，100% 的患者（全部 17 名患者）的血清磷水平在 4-21 天内恢复正常，90% 的患者（10 名患者中有 9 名）的 FGF-23 水平下降。我们发现 64.7% 的肿瘤（17 个肿瘤中的 11 个）是磷酸盐间质肿瘤或磷酸盐间质肿瘤混合结缔组织变体。检测疑似 TIO 患者的血清磷和 FGF-23 水平对于诊断 TIO 至关重要。 F-18-FDG PET/CT、(99)mTc-OCT 闪烁扫描和体检在揭示 TIO 相关肿瘤方面发挥着相当大的作用。 TIO 相关肿瘤更常见于下肢，而不是其他部位；因此，需要仔细检查下肢。完全手术切除可使实验室检查参数正常化并缓解 TIO 患者的症状。
Tumor-induced osteomalacia (TIO) is a rare acquired form of hypophosphatemic osteomalacia, which is usually attributed to the overproduction of fibroblast growth factor 23 (FGF-23) by benign mesenchymal neoplasms. Localization and thereafter surgical resection of tumors lead to a cure. The present study aimed to investigate the clinical data, diagnostic methods, and follow-up after tumor resection at one medical center in Shanghai to characterize the profile of this rare disorder and to share our successful experience in diagnosis and treatment. Twentythree patients with adult-onset hypophosphatemia osteomalacia seen in Shanghai Sixth People's Hospital from 2009 to 2014 and 95 normal individuals were enrolled. After taking a medical history and performing a physical examination, we analyzed the laboratory results (including the serum FGF-23 levels) and localized the tumors by 18F-fluorodeoxyglucose positron emission tomography and computed tomography (18F-FDG PET/CT), (99)mT-coctreotide ((99)mT-c-OCT) scintigraphy, and magnetic resonance imaging (MRI). On the basis of the results of laboratory tests and imaging findings, tumor resection was conducted in 17 patients with a certain diagnosis of TIO. The results demonstrated that the 17 patients (nine men and eight women, average age 46.6 +/- 12.9 years) had TIO. FGF-23 level was elevated in 94.1 % of patients (16 of 17 patients). Serum phosphorus level decreased in 100 % of patients. F-18-FDG PET/CT revealed five tumors, (99)mT-cOCT scintigraphy revealed two tumors, physical examination revealed nine tumors, and MRI revealed one tumor, among which 58.8 % of the causative tumors (10 of 17 tumors) were located in the lower extremities. After tumor resection, serum phosphorus levels normalized in 100 % of patients (all 17 patients) in 4-21 days and FGF-23 levels decreased in 90 % of patients (nine of ten patients). We found 64.7 % of the tumors (11 of 17 tumors) were phosphaturic mesenchymal tumors or a phosphaturic mesenchymal tumor mixed connective tissue variant. Measurement of serum phosphorus and FGF-23 levels in patients with suspected TIO is of paramount importance for diagnosing of TIO. F-18-FDG PET/ CT, (99)mTc-OCT scintigraphy, and physical examination play a considerable role in revealing TIO-associated tumors. TIO-associated tumors were more frequently located in the lower extremities than in other places; thus, the lower extremities need to be carefully checked. Complete surgical resection results in normalization of parameters in laboratory tests and relief of symptoms of TIO patients.