目的:肿瘤诱导的骨软化症(TIO)是由成纤维细胞生长因子23(FGF23)过度产生引起的罕见病理。其常见临床特征包括全身性肌无力,骨痛,和骨折。完全切除有问题的肿瘤是主要的治疗方法。在这份报告中,我们介绍了使用液氮处理的荷瘤自体移植物治疗的FGF23产生肿瘤的第一例TIO。
方法:一名63岁女性出现全身疼痛,尤其是左臂。该患者被诊断患有产生FGF23的左肱骨肿瘤。使用用液氮处理的荷瘤自体移植物进行所涉及肿瘤的广泛切除。术后,FGF23和碱性磷酸酶(ALP)水平显着降低,无机磷酸盐恢复正常。随后还缓解了全身疼痛。手术后立即,开始左肩和肘部的活动范围。指示患者在旋转约束下进行前屈和外展至90°。在术后12个月观察到几乎完全的骨愈合。术后功能结果如下:肌肉骨骼肿瘤协会(MSTS)评分为27/30,90%,国际保肢协会(ISOLS)评分为26/30,87%。手术十年后,根据X光片,截骨线完全模糊.患者无疾病且无活动限制。
结论:这是首例FGF23产生肿瘤的广泛切除和使用带有肿瘤的冷冻自体移植物进行重建的病例报告,结果极佳。
OBJECTIVE: Tumor-induced osteomalacia (TIO) is a rare pathology caused by overproduction of fibroblast growth factor 23 (FGF23). Its common clinical features include generalized muscle weakness, bone pain, and fractures. Complete resection of the offending tumor is the mainstay treatment. In this report, we present the first case of TIO by an FGF23 producing tumor treated using a tumor-bearing autograft treated with liquid nitrogen.
METHODS: A 63-year old female presented with generalized body pain, particularly in the left arm. The patient was diagnosed with a FGF23 producing tumor of the left humerus. Wide resection of the involved tumor was performed using a tumor-bearing autograft that was treated with liquid nitrogen. Postoperatively, the FGF23 and alkaline phosphatase (ALP) levels significantly decreased and inorganic phosphate normalized. There was also subsequent relief of generalized body pain. Immediately after the operation, range of motion of the left shoulder and elbow was initiated. The patient was instructed to perform forward flexion and abduction up to 90° with a rotational restraint. Almost complete bone union was observed at 12 months post procedure. Postoperative functional results were as follows: Musculoskeletal Tumor Society (MSTS) score of 27/30, 90% and International Society of Limb Salvage (ISOLS) score of 26/30, 87%. Ten years after the surgery, osteotomy line was completely obscured based on radiographs. The patient was disease free and without activity limitation.
CONCLUSIONS: This is the first case report of wide excision of a FGF23 producing tumor and reconstruction using a tumor-bearing frozen autograft performed with excellent outcomes.