目的 通过对肿瘤性骨软化症(tumor-induced osteomalacia ,TIO)患者临床特点、诊断过程及术后疗效随访的分析,提高对TIO的认识及临床诊治水平。方法 回顾性分析15例TIO确诊患者的临床特点及手术前后临床及生化指标变化。结果 15例年龄(42.8±10.7)岁,病程为(3.5±2.8)年;其中2例复发(13.3%),1例复发后恶变(6.7%)。血磷(0.41±0.10)mmol/L、肾磷酸根阈值( RTPC)(0.33±0.10)mmol/L,均低于正常值。15例共住院手术19人次,肿瘤位于骨组织10人次,软组织9人次,13例手术一次性治愈(86.7%)。19次术后病理结果均为磷酸盐尿性间叶细胞瘤混合结缔组织型(PMTMCT)。分析显示:肿瘤位于软组织患者较肿瘤位于骨组织患者血磷及RTPC均更低(t=2.264,P=0.041);血磷主要受RTPC影响(标准系数0.836,t=3.862,P=0.005);RTPC受肿瘤体积、肿瘤组织来源相关影响(标准系数0.405、0.546,t=-2.635、3.925,P=0.030、0.004)。结论 对于TIO的患者应警惕恶性及复发的可能;RTPC能较24 h尿磷更好地反映肾小管重吸收磷的受损情况;肿瘤位于软组织患者较肿瘤位于骨组织病情更为严重。
Abstract
Objective To investigate the clinical features, diagnostic process and outcomes of tumor-induced osteomalacia (TIO).Methods A retrospective analysis of fifteen patients with TIO was performed, involving pre- and post-operative clinical features and biochemical changes.Results Among the fifteen patients, there were two cases of recurrence (13.3%)and one case of metastases (6%)after recurrence. The average age was (42.8±10.7 )years, and the mean duration of disease was (3.5±2.8 )years. Serum phosphorus concentrations were low in all these patients (0.41±0.10)mmol/L.The renal phosphate threshold (RTPC) decreased in all the patients with an average of (0.33±0.10) mmol/L. These patients were treated surgically nineteen times. Nine tumors were found in soft tissues and ten tumors in bones. Thirteen of these cases (86.7%) were cured through primary operation and serum phosphorus concentrations returned to normal in (3.7±1.6 )days. All the nineteen tumors were phosphaturic mesenchymal tumors of the mixed connective tissue variant. One case was found to be malignant during the follow-up. Compared with tumors in bones, tumors in soft tissues caused a lower serum phosphorus concentration and lower RTPC (t=2.264, P=0.041). Regression analysis showed that the blood phosphorus concentration was influenced primarily by RTPC (standardized beta coefficient=0.836, t=3.862,P=0.005), and that RTPC was influenced by tumor volume and tumor tissue origin (standardized beta coefficient=0.405、0.546,t=-2.635、3.925,P=0.030、0.004, respectively).Conclusions The chance of recurrence and metastasis of TIO is high. Compared with 24-hour urine phosphorus concentrations, RTPC is more suitable for the evaluation of impaired renal tubular function. Patients with a tumor in soft tissues are in more critical condition.
关键词
肿瘤性骨软化症 /
低磷血症 /
恶性 /
复发 /
磷酸盐尿性间叶细胞瘤
Key words
tumor-induced osteomalacia /
malignancy /
recurrence /
hypophosphatemia /
phosphaturic mesenchymal tumor
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基金
北京市优秀人才培养资助青年骨干个人项目(2017000021469G260)
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