Clinical characteristics and treatment strategy of five phosphaturic mesenchymal tumors in hips
10.3760/cma.j.issn.0253-2352.2018.10.005
- VernacularTitle:髋部磷酸盐尿性间叶瘤5例临床特点及治疗策略
- Author:
Da CHEN
1
;
Tianan GUAN
;
Jingli XU
;
Yaohua HUANG
;
Zhihui PANG
;
Haibin WANG
;
Yirong ZENG
;
Qingwen ZHANG
;
Wei HE
;
Bin FANG
Author Information
1. 510405,广州中医药大学
- Keywords:
Oncogenic osteomalacia;
Hypophosphatemia;
Hip;
Bone fractures
- From:
Chinese Journal of Orthopaedics
2018;38(10):601-606
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical characteristics and treatment strategy of phosphaturic mesenchymal tumors in hips.Methods From May 2017 to November 2017,five patients with phosphaturic mesenchymal tumors in hips,who treated in our hospital were retrospectively reviewed.The clinical manifestations,laboratory inspection,radiological examination,pathological examination and treatment were analyzed.Results Two male cases (40％) and 3 female (60％) were included.The overall age was 49～63 years old (average 54.40±5.37 years old).The course was 19～101 months (average 51.20±32.41months).Four cases of tumor were located in femoral head and 1 case was in femur intertrochanteric region.The maximum tumor diameter was 0.76～1.83 cm (average 1.28±0.39 cm).The early clinical manifestations of the patients were mainly non-specific bone pain or fatigue.The symptoms of the hip were not obvious.All patients had been misdiagnosed.After pathological frac-ture of the hip,the patient suffered from hip pain,thigh pain,fatigue,etc.,or limited hip function.The severe patients had a systemic multiple pathologic fractures.The serum phosphorus was lower than normal in preoperative period and recovered to normal level in 3-8 days after surgery.The postoperative ALP decreased significantly than preoperative in 4 patients and 1 case was slightly higher than preoperative.Preoperative 1,25-(OH)2-D3 and PTH were in normal range.99Tcm-octreotide (OCT) scan or 68Ga DOTA-TATE PET/CT can detect the disease.X-ray,CT and MRI can identify the lesions.The lesions of femoral head were basically under the joint surface,with a nodular change.Part of the tumor was infiltrating and close to the cortex.The pathology was mainly manifested as a large number of thin-walled vessels in the bone trabeculae.There were dense spindle cells or astrocytes between the blood vessels and the atypia is not obvious.Three patients were treated with total hip arthroplasty and two with segmental resection and bone graft.After surgical removal of the tumor,the patient's systemic pain or anemic symptoms were gradually relieved and the joint function was restored.Meanwhile,the bone density was increased.Conclusion The clinical features of the phosphaturic mesenchymal tumors in hips were not obvious.Comprehensive diagnosis should be carried out in combination with the clinical manifestations,laboratory examination,radiological examination and pathological examination.Total hip arthroplasty or segmental resection with bone graft can effectively remove the tumor and achieve good prognosis.
