肿瘤引起的骨软化:一种新发的副肿瘤综合征

Guillermo Alonso , Mariela Varsavsky
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引用次数: 7

摘要

内分泌副肿瘤综合征是某些肿瘤的远处表现。一种罕见但越来越多报道的形式是肿瘤诱导的骨软化症,这是一种与肿瘤分泌成纤维细胞生长因子23(FGF-23)有关的低磷血症。这种疾病的主要生化表现包括低磷血症、肾小管对磷酸盐的再吸收不适当地低或正常、血清骨化三醇水平低、血清碱性磷酸酶水平升高以及血清FGF-23水平升高或正常。这些肿瘤通常较小、良性、生长缓慢且难以发现,主要局限于四肢软组织。组织学上,混合结缔组织型的磷尿质间充质肿瘤最为常见。已经提出了各种成像技术,结果各不相同。治疗的选择是肿瘤的全手术切除。药物治疗包括口服磷和骨化三醇补充剂、奥曲肽、西那卡司和单克隆抗体。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Tumour-induced osteomalacia: An emergent paraneoplastic syndrome

Endocrine paraneoplastic syndromes are distant manifestations of some tumours. An uncommon but increasingly reported form is tumour-induced osteomalacia, a hypophosphatemic disorder associated to fibroblast growth factor 23 (FGF-23) secretion by tumours. The main biochemical manifestations of this disorder include hypophosphatemia, inappropriately low or normal tubular reabsorption of phosphate, low serum calcitriol levels, increased serum alkaline phosphatase levels, and elevated or normal serum FGF-23 levels. These tumours, usually small, benign, slow growing and difficult to discover, are mainly localized in soft tissues of the limbs. Histologically, phosphaturic mesenchymal tumours of the mixed connective tissue type are most common. Various imaging techniques have been suggested with variable results. Treatment of choice is total surgical resection of the tumour. Medical treatment includes oral phosphorus and calcitriol supplements, octreotide, cinacalcet, and monoclonal antibodies.

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