12例肿瘤性骨软化症的临床诊治及术后随访  

Clinical management and postoperative follow up of 12 patients with tumor-induced osteomalacia

作　　者：巴建明[1] 桑艳红[2] 陆菊明[1] 母义明[1] 窦京涛[1] 吕朝晖[1] 王先令[1] 杨国庆[1] 欧阳金枝[1] 杜锦[1] 郭清华[1] 谷伟军[1] 金楠[1] 

机构地区：[1]解放军总医院内分泌科,北京100853 [2]郑州大学第五附属医院内分泌科

出　　处：《中华内分泌代谢杂志》2011年第1期19-23,共5页Chinese Journal of Endocrinology and Metabolism

摘　　要：目的 通过对肿瘤性骨软化症（tumor-induced osteomalacia,TIO）患者临床特点、诊断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 本院自2004年至2010年4月间收治确诊的TIO患者12例.分析患者的临床特点、诊断、治疗及手术前后临床及生化指标变化.结果 12例中男性7例,女性5例,平均年龄（41.8±9.6）岁（20～56岁）,病程2～14年,中位病程4.0年.12例患者均有骨痛、肌无力及活动障碍.12例患者血磷均呈明显低下,范围0.30～0.56 mmol/L,血钙正常或轻度降低,碱性磷酸酶除1例外均有不同程度的升高,甲状旁腺素（PTH）5例正常,7例轻度升高.本组患者有9例行99mTc-奥曲肽扫描（99mTc-Oct）,发现头部（1例）,躯干（1例）及四肢不同部位（7例）生长抑素受体高表达病变,另3例患者经查体发现右腰背、左大腿内侧、足底软组织肿物.12例患者均行手术治疗,经病理证实有6例为血管内皮细胞瘤或纤维血管瘤,4例为腱鞘巨细胞瘤或纤维瘤,1例为脂肪肉瘤,1例为尿磷性间叶性肿瘤.手术切除肿瘤后10例血磷恢复正常,患者术后随访2～64个月,血磷正常,骨痛及肌无力均显著改善,可正常行走及生活.结论 加强在低磷软骨病患者中TIO的筛查,采用99mTc-Oct及其他影像检查可有效定位肿瘤,手术治疗去除病灶可使血磷恢复正常,临床症状改善、消失,取得良好的治疗效果.Objective To better understand the clinical management of tumor-induced osteomalacia （TIO） by analyzing the clinical features, diagnosis, treatment, postoperative biochemical changes, and clinical status in 12 cases of TIO. Methods Twelve cases of TIO hospitalized from 2004 to April 2010 were reviewed retrospectively. All cases were diagnosed based on their clinical manifestation, hypophosphatemia, and image study including technetium-99m octreotide scintigraphy （99mTc-Oct）. Resuits There were 7 males and 5 females with mean age of （41.8±9.6） years （20 to 56 years）. The course of disease was from 2 to 14 years （ median course 4.0 years）. They all presented with bone pain, gait disturbance, muscle pain, and muscle weakness. Serum phosphate（ Pi）levels were low in 12 cases with a range from 0.30 to 0.56 mmol/L. 99mTc-Oct was performed in 9 cases and it showed that the lesions were located in head of femur, fibula, retrocalcaneal area, foot, humerus,metacarpal, posterior chest wall or near nasal bone （apex partis petrosae ossis temporalis）. Subcutaneous soft tissue mass was found in another 3 cases at loin, thigh, and foot by physical examination. The tumors were confirmed by CT, MRI or ultrasonography. Twelve patients underwent operation to remove the tumors and histopathology showed hemangioendothelioma or fibrous angioma （6 cases）, giant cell tumor or fibroma of tendon sheath（4 cases）, liposarcoma（1case）, and phosphaturic mesenchymal tumor（1case）. Serum Pi levels returned to normal in 10 patients after resection of tumor. During 2 to 64 months follow up, symptoms of bone pain and muscle weakness were improved obviously. Conclusions Patients with hypophosphatemic osteomalacia should be thoroughly investigated for TIO. 99mTc-Oct and other imaging examinations can effectively locate the tumors. Once the hidden tumor is found and excised, the patient will recover and enjoy normal life with normalized Pi concentrations and marked improvement of symptoms.

关 键 词：肿瘤性骨软化症 低磷血症 奥曲肽扫描 间叶组织肿瘤 

分 类 号：R681[医药卫生—骨科学]