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[肿瘤定位与肿瘤诱导性骨软化症的治疗]

[Tumor localization and treatment of tumor-induced osteomalacia].

作者信息

Beil Frank Timo, Stürznickel Julian, Rolvien Tim, Amling Michael, Oheim Ralf

机构信息

Lehrstuhl für Orthopädie, Klinik und Poliklinik für Unfallchirurgie und Orthopädie, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland.

Institut für Osteologie und Biomechanik, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland.

出版信息

Z Rheumatol. 2022 Apr;81(3):182-188. doi: 10.1007/s00393-022-01160-1. Epub 2022 Feb 1.

Abstract

Tumor-induced osteomalacia (TIO) or oncogenic osteomalacia (OOM) is a rare paraneoplastic renal phosphate wasting syndrome. The disease is mostly triggered by small, benign mesenchymal tumors that express somatostatin receptors (SSTR) and produce excessive levels of fibroblast growth factor 23 (FGF 23) or other phosphatonins. These reduce the phosphate back resorption in the proximal tubules of the kidneys, thereby causing hypophosphatemia and lead to an absolute or relatively low calcitriol serum concentration. The main symptoms include muscle weakness, bone pain and recurrent insufficiency fractures secondary to sometimes pronounced osteomalacia. The suspected diagnosis can only be confirmed by determination of the phosphate level. It can often take years before the tumor is successfully localized. The necessary tumor localization is often the most difficult step in the treatment before the OOM can be curatively treated by open surgical resection of the tumor. In recent years new approaches for faster tumor localization and treatment of the tumor have been developed. Positron emission tomography (PET) in co-registration with computed tomography (Ga-DOTA-TATE PET/CT) is currently the most sensitive imaging methodology for tumor detection. The application of the monoclonal FGF 23 antibody burosumab represents a promising new option in the treatment of inoperable adult OOM.

摘要

肿瘤诱导的骨软化症(TIO)或致癌性骨软化症(OOM)是一种罕见的副肿瘤性肾性磷酸盐消耗综合征。该疾病主要由表达生长抑素受体(SSTR)并产生过量成纤维细胞生长因子23(FGF 23)或其他磷调节素的小的良性间充质肿瘤引发。这些物质会减少肾脏近端小管中的磷酸盐重吸收,从而导致低磷血症,并导致血清骨化三醇浓度绝对或相对降低。主要症状包括肌肉无力、骨痛以及有时因明显的骨软化症继发的反复性不全骨折。疑似诊断只能通过测定磷酸盐水平来确诊。通常需要数年时间才能成功定位肿瘤。在通过开放性手术切除肿瘤对OOM进行根治性治疗之前,必要的肿瘤定位往往是治疗中最困难的一步。近年来,已经开发出了用于更快肿瘤定位和治疗肿瘤的新方法。正电子发射断层扫描(PET)与计算机断层扫描联合(镓-多胺基多乙酸-奥曲肽PET/CT)目前是检测肿瘤最敏感的成像方法。单克隆FGF 23抗体布罗索尤单抗的应用是治疗无法手术的成人OOM的一种有前景的新选择。

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