肿瘤源性骨软化症的临床特征

目的分析肿瘤源性骨软化症(tumor-induced osteomalacia,TIO)患者的临床特点、诊断、病理及手术后情况,以提高临床对TIO的认识.方法收集并整理2008年1月至12月于解放军总医院就诊的14例肿瘤源性骨软化症患者的病史、一般情况、临床表现、辅助检查、病理及术后情况.结果患者平均病程为(3.0±3.8)年,临床症状以骨痛(14/14,100%)和乏力(6/14,42.9%)最常见,部分患者有病理性骨折,在确诊前均有误诊病史.生化特点以低血磷(0.43±0.12)mmol/L、高尿磷(13.71±5.32)mmol/L 24 h、血碱性磷酸酶升高(309.30±146.41)U/L和正常血钙(2.19±0.13 mmol/L)为主.定位诊断中,99Tcm-奥曲肽SPECT/CT扫描和68 Ga-DOTA-TATE PET/CT阳性率均较高(100%),并经相应部位的CT、MRI或超声检查证实.肿瘤分布在全身不同部位的软组织(4/14,28.6%)或骨组织(10/14,71.4%)中.手术切除肿瘤后,血磷平均恢复时间为(6.4±2.3)d.1例因病灶难以完全切除,术后未缓解.2例患者术后出现复发.结论TIO起病隐匿,症状不具有特异性,容易被误诊.99 Tcm-奥曲肽SPECT/CT扫描或68 Ga-DOTATATE PET/CT检查对定位诊断效率较高.手术治疗可以治愈TIO,但远期仍可能复发,需要定期随访.

Clinical features of tumor-induced osteomalacia

Objective To analyze the clinical characteristics,diagnosis,pathology,and post-operative condi-tions of tumor-induced osteomalacia in order to improve the understanding of the disease.Methods Fourteen patients,who were diagnosed as tumor-induced osteomalacia in PLA General Hospital from January 2008 to December 2018,were collected and sorted out,including their medical history,general situation,clinical manifestations,auxiliary examina-tions,pathology and postoperative conditions.Results The average time from onset to a correct diagnosis was(3.0±3.8)years.Bone pain(14/14,100%)and fatigue(6/14,42.9%)were the most common clinical symptoms.Every patient had a history of misdiagnosis.The main biochemical characteristics were low serum phosphorus(0.43±0.12)mmol/L,high urine phosphorus(13.71±5.32)mmol/24 h,elevated serum alkaline phosphatase(309.30±146.41)U/L,and normal serum calcium(2.19±0.13)mmol/L.99 Tcm-octreotide SPECT/CT scan and 68 Ga-DOTA-TATE PET/CT were helpful in locating the tumors.CT,MRI,or ultrasonography were also very helpful.Tumors were found in soft tissue(4/14,28.6%)or bone tissue(10/14,71.4%)in different parts of the body.The average recovery time of blood phosphorus was(6.4±2.3)days after operation.One patient didn t relieved after surgery because the tumor was difficult to be completely resected.Two patients had recurrence after operation.Conclusions Tumor-related osteoma-lacia is easily to be misdiagnosed.For tumor localization,99Tcm-octreotide SPECT/CT scan and 68Ga-DOTATATE PET/CT scan are recommended.CT,MRI and Ultrasound examinations are helpful in localization.Tumor resection is the first treatment choice.The recovery of blood phosphorus is a sign of successful operation.However,recurrence may occur in the long term,and regular follow-up is needed.