Tumour-induced软骨病。

Athira Ramakrishanan, Aashish Parekh, Shankarmurthy Gayana, Shanthi Velusamy, Abhilasha Sadhoo
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引用次数: 0

摘要

肿瘤诱导的骨软化症(TIO)是一种罕见的副肿瘤综合征,由成纤维细胞生长因子23 (FGF-23)的产生增加引起。一名45岁男性患者向我们报告了2年的进行性虚弱,并伴有复发性骨折,创伤很小。他的FGF-23高于正常范围,DOTATATE正电子发射断层扫描(PET)显示软组织密度明显增强,包括左侧后筛,延伸到蝶筛隐窝和蝶窦口。他接受了内窥镜鼻窦手术并切除了肿瘤。组织病理学检查显示为磷质间充质瘤-混合结缔组织型。术后血清磷水平从第1天开始升高,第3天达到正常值2.5 mg/dl。出院后继续口服钙和维生素D3。他的肌痛逐渐改善,在接下来的1个月里他开始独立行走。从症状出现到治疗的平均延迟时间为2年零5个月。及时的诊断和细致的随访是治疗这种罕见疾病的必要条件。
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.

Tumour-induced osteomalacia (TIO) is a rare paraneoplastic syndrome caused by increased production of fibroblast growth factor 23 (FGF-23). A 45-year-old man presented to us with progressive weakness over 2 years along with recurrent fractures with minimal trauma. His FGF-23 was found to be above the normal range and DOTATATE positron emission tomography (PET) scan showed a well-defined enhancing soft tissue density involving the left posterior ethmoid with extension to the spheno-ethmoidal recess and sphenoid sinus ostium. He underwent endoscopic sinus surgery and excision of the tumour. Histopathological examination showed features of phosphaturic mesenchymal tumour-mixed connective tissue type. Postoperatively the serum phosphorus level increased from day 1 and reached the normal value of 2.5 mg/dl on day 3. He was discharged and continued on oral calcium and vitamin D3. Gradually his myalgia improved and he started walking independently over the next 1 month. The mean delay from symptom onset to treatment in our patient was 2 years and 5 months. Timely diagnosis and meticulous follow-up are necessary for the management of patients with this rare disorder.

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