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[迟发性磷尿性间充质肿瘤所致肿瘤性骨软化症]

[Tumor-induced osteomalacia caused by a late-revealing phosphaturic mesenchymal tumor].

作者信息

Chazal T, Khanine V, Lidove O, Godot S, Ziza J-M

机构信息

Service de médecine interne et rhumatologie, groupe hospitalier diaconesses Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France.

Service de médecine interne et rhumatologie, groupe hospitalier diaconesses Croix-Saint-Simon, 125, rue d'Avron, 75020 Paris, France.

出版信息

Rev Med Interne. 2017 Jun;38(6):412-415. doi: 10.1016/j.revmed.2016.08.020. Epub 2016 Sep 19.

Abstract

INTRODUCTION

Osteomalacia is associated with diffuse pain and multiple fractures and therefore, diagnosis and treatment of this condition are necessary. Clinicians should be aware of an uncommon mechanism of osteomalacia where hypophosphataemia is secondary to renal phosphaturia because of the production by a mesenchymal phosphaturic tumor of FGF-23. This tumor should be localized and removed to cure this tumor-induced osteomalacia.

OBSERVATION

A 70-year-old female patient was admitted to explore diffuse pain caused by multiple fractures secondary to osteomalacia. Despite vitamin D supplementation, she remained profoundly hypophosphoremic with major renal phosphaturia. A tumor-induced mechanism was suspected because of high level of FGF-23. It took more than three years of investigation to spot the causal phosphaturic mesenchymal tumor despite annual repetition of indium-labelled scintigraphy and PET-scan. The resection of the tumor, located between two phalanges of the right foot, cured the patient with sustained normal rate of serum level of phosphorus after two years.

CONCLUSION

Tumor-induced osteomalacia is a diagnostic challenge because the localization of the tumor may be a long process. Patients should be monitored clinically and imaging studies repeated until a diagnosis is made and the causal tumor removed.

摘要

引言

骨软化症与弥漫性疼痛和多发性骨折相关,因此,对这种疾病进行诊断和治疗很有必要。临床医生应了解一种不常见的骨软化症机制,即由于间充质磷尿性肿瘤产生成纤维细胞生长因子23(FGF - 23),导致低磷血症继发于肾性磷尿。应定位并切除该肿瘤以治愈这种肿瘤诱导的骨软化症。

观察

一名70岁女性患者因骨软化症继发多发性骨折引起的弥漫性疼痛入院。尽管补充了维生素D,但她仍严重低磷血症且有严重的肾性磷尿。由于FGF - 23水平高,怀疑是肿瘤诱导机制。尽管每年重复进行铟标记闪烁扫描和PET扫描,但经过三年多的检查才发现导致磷尿的间充质肿瘤。切除位于右脚两个趾骨之间的肿瘤后,患者在两年后血清磷水平持续正常,得以治愈。

结论

肿瘤诱导的骨软化症是一项诊断挑战,因为肿瘤定位可能是一个漫长的过程。应临床监测患者并重复进行影像学检查,直到做出诊断并切除致病肿瘤。

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