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颅底肿瘤所致骨软化症的确定性手术治疗及血清成纤维细胞生长因子23半衰期的测定

Definitive surgical treatment of osteomalacia induced by skull base tumor and determination of the half-life of serum fibroblast growth factor 23.

作者信息

Hana Taijun, Tanaka Shota, Nakatomi Hirofumi, Shojima Masaaki, Fukumoto Seiji, Ikemura Masako, Saito Nobuhito

机构信息

Department of Neurosurgery, Faculty of Medicine, The University of Tokyo Hospital, Tokyo, Japan.

Department of Endocrinology, Faculty of Medicine, The University of Tokyo Hospital, Tokyo, Japan.

出版信息

Endocr J. 2017 Oct 28;64(10):1033-1039. doi: 10.1507/endocrj.EJ17-0177. Epub 2017 Aug 2.

Abstract

Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome often associated with fibroblast growth factor 23 (FGF23)-producing tumors such as phosphaturic mesenchymal tumor, mixed connective tissue variant (PMTMCT) affecting the bone and soft tissue. We experienced a patient with progressive bone and muscle pain due to FGF23-related TIO. Venous sampling had strongly suggested the anterior skull base as a source of FGF23, which led to the discovery of a small tumor in the ethmoid sinus extending intracranially. Radical surgical resection confirmed the histological diagnosis of PMTMCT with FGF23 immunopositivity and achieved durable tumor control with complete resolution of symptoms. We serially measured serum FGF23 level before, during and after surgery and analyzed the data to determine the half-life of FGF23. Serum FGF23 level sharply declined as early as 20 minutes after en bloc tumor resection and completely normalized after surgery. The half-life of FGF23 was calculated to be approximately 18.5 minutes using single phase exponential decay model as well as semilog transformation formula. Serial measurements of serum FGF23 level can potentially declare "complete" resection of a FGF23-producing tumor and total cure of TIO; in this regard, development of its intraoperative measurement would be helpful in the management of this endocrine tumor.

摘要

肿瘤诱导的骨软化症(TIO)是一种罕见的副肿瘤综合征,常与产生成纤维细胞生长因子23(FGF23)的肿瘤相关,如影响骨骼和软组织的磷酸尿性间叶肿瘤、混合结缔组织变体(PMTMCT)。我们遇到一名因FGF23相关的TIO而出现进行性骨痛和肌肉疼痛的患者。静脉采血强烈提示前颅底是FGF23的来源,这导致在筛窦发现一个向颅内延伸的小肿瘤。根治性手术切除证实了PMTMCT的组织学诊断,FGF23免疫阳性,并通过症状完全缓解实现了持久的肿瘤控制。我们在手术前、手术期间和手术后连续测量血清FGF23水平,并分析数据以确定FGF23的半衰期。血清FGF23水平在整块肿瘤切除后20分钟就急剧下降,术后完全恢复正常。使用单相指数衰减模型以及半对数转换公式计算出FGF23的半衰期约为18.5分钟。血清FGF23水平的连续测量可能有助于判定产生FGF23的肿瘤是否“完全”切除以及TIO是否完全治愈;在这方面,术中测量方法的开发将有助于这种内分泌肿瘤的管理。

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