Wang Hai, Zhong Dingrong, Liu Yong, Jiang Yan, Qiu Guixing, Weng Xisheng, Xing Xiaoping, Li Mei, Meng Xunwu, Li Fang, Zhu Zhaohui, Yu Wei, Xia Weibo, Jin Jin
Departments of Orthopaedic Surgery (H.W., Y.L., G.Q., X.W., and J.J.), Endocrinology (Y.J., X.X., M.L., X.M., and W.X.), Pathology (D.Z.), Nuclear Medicine (F.L. and Z.Z.), and Radiology (W.Y.), Peking Union Medical College Hospital (PUMCH), Number 1 Shuaifuyuan hutong, Beijing 100730, People's Republic of China. E-mail address for H. Wang:
J Bone Joint Surg Am. 2015 Jul 1;97(13):1084-94. doi: 10.2106/JBJS.N.01299.
Tumor-induced osteomalacia is a rare and fascinating paraneoplastic syndrome usually caused by a small, benign phosphaturic mesenchymal tumor. Most tumors are treated surgically, but we are unaware of any reports that compare the results of curettage and segmental resection for lesions in long bones.
Seventeen patients (ten male and seven female) with tumor-induced osteomalacia lesions in long bones, who underwent surgical treatment from December 2004 to August 2013 in our hospital, were included in this retrospective study. The mean follow-up (and standard deviation) was 35 ± 27 months (range, twelve to 116 months). The characteristics of the tumor and the effects of different surgical treatments (curettage compared with segmental resection) were evaluated.
All patients showed typical clinical characteristics of tumor-induced osteomalacia, including elevated serum fibroblast growth factor-23 (FGF-23); 82% of tumors were in the epiphysis, and 82% grew eccentrically. The mean maximum diameter of the tumors was 2.4 ± 2.0 cm. The complete resection rates were similar for curettage (67%) and segmental resection (80%). However, the recurrence rate after curettage (50%) was higher than that after segmental resection (0%). The complete resection rate for secondary segmental resection (75%) was not different from that for primary segmental resection (83%). All of our cases of tumor-induced osteomalacia were caused by phosphaturic mesenchymal tumors. After successful removal of tumors, serum FGF-23 returned to normal within twenty-four hours and serum phosphorus levels returned to normal at a mean of 6.5 ± 3.5 days.
Most lesions in long bones are located in the epiphysis, so curettage is first suggested to maintain joint function. If curettage is incomplete or there is a recurrence, secondary segmental resection should be considered curative. Changes of serum FGF-23 and phosphorus levels before and after the operation may be of prognostic help.
肿瘤性骨软化症是一种罕见且引人关注的副肿瘤综合征,通常由一种小型良性磷酸尿性间叶肿瘤引起。大多数肿瘤通过手术治疗,但我们未发现任何比较长骨病变刮除术和节段性切除术结果的报告。
本回顾性研究纳入了2004年12月至2013年8月在我院接受手术治疗的17例长骨肿瘤性骨软化症病变患者(10例男性,7例女性)。平均随访时间(及标准差)为35±27个月(范围为12至116个月)。评估了肿瘤的特征以及不同手术治疗(刮除术与节段性切除术)的效果。
所有患者均表现出肿瘤性骨软化症的典型临床特征,包括血清成纤维细胞生长因子23(FGF - 23)升高;82%的肿瘤位于骨骺,82%呈偏心生长。肿瘤的平均最大直径为2.4±2.0厘米。刮除术(67%)和节段性切除术(80%)的完整切除率相似。然而,刮除术后的复发率(50%)高于节段性切除术后(0%)。二次节段性切除术的完整切除率(75%)与初次节段性切除术(83%)无差异。我们所有的肿瘤性骨软化症病例均由磷酸尿性间叶肿瘤引起。成功切除肿瘤后,血清FGF - 23在24小时内恢复正常,血清磷水平平均在6.5±3.5天恢复正常。
大多数长骨病变位于骨骺,因此首先建议行刮除术以维持关节功能。如果刮除术不彻底或出现复发,应考虑二次节段性切除术作为根治性治疗。手术前后血清FGF - 23和磷水平的变化可能有助于判断预后。