肿瘤源性骨软化症责任肿瘤的CT和MRI表现

CT and MRI Manifestations of TIO Tumors

目的探讨肿瘤源性骨软化症(TIO)责任肿瘤的CT和MRI表现,以提高对此疾病的诊断水平。方法回顾性分析经临床、实验室检查、影像学及病理学确诊的23例TIO患者的影像及临床资料。结果 23例TIO患者,男18例,女5例,年龄17~61岁,中位年龄39.0岁,病程1~11年,平均4.4年;病变最大径0.6~8.0 cm,平均2.8 cm;病变发生于股骨头5例,股骨颈3例,股骨下段3例,腓骨2例,下颌骨2例,上颌骨1例,颧弓1例,筛窦2例,鼻腔1例,坐骨直肠窝1例,膝关节内侧软组织1例,L5水平椎管内1例。21例有手术病理结果,其中16例诊断为磷酸盐尿性间叶肿瘤(1例为低度恶性,其余均为良性),5例因未做免疫组织化学检测仅诊断为梭形细胞肿瘤。11例行CT扫描,病变大部分密度均匀,部分有斑点状钙化,2例病变含有脂肪密度,所有病例未见明显出血、坏死及囊变;CT值-16~107 HU,平均为41.0 HU,仅1例增强CT表现为明显均匀强化;骨内及颌面部病变边界清晰,软组织内病变边界模糊,骨皮质旁病变共8例,其中7例可见邻近骨皮质破坏。19例行MRI扫描,病变在T_1WI上均为低信号,软组织内病变T_2WI上为高信号,骨内及骨皮质旁病变T_2WI抑脂像上为高信号、低信号或高低混杂信号,钆对比剂(Gd-DTPA)增强均表现为明显强化。结论由于TIO肿瘤病理组织形态上的多样性,决定了其影像学表现有较大差异,仅依靠影像学定性诊断非常困难。但是CT和MRI均可发现及清晰显示病变,对于已经排除其他病因并且无家族史的成年低磷骨软化患者,CT和MRI的异常影像学发现可以做出提示性诊断,也可以用于TIO确诊者的术前评估和治疗随访。

Objective To investigate the CT and MRI features of tumor causing TIO,and to improve the diagnostic level of the disease. Methods CT and MRI images as well as clinical data of TIO patients diagnosed by clinical,laboratory examination,imaging and pathology were analyzed retrospectively. Results There were 23 cases,18 male cases,5 female cases,aged 17-61 years old,median age was 39. 0 years,duration of 1-11 years,an average of 4. 4 years; the maximum lesion diameter 0. 6-8. 0 cm,average 2. 8 cm. Lesion location： 5 cases of femoral head,femoral neck in 3 cases,distal femur in 3 cases,fibula（ 2 cases）,2 cases of jaw,maxilla 1 case,1 case of zygomatic arch,ethmoid sinus in 2 cases,1 case of nasal cavity,ischiorectal fossa（ n = 1）,medial knee soft tissue in 1 case,lumbar 5 spinal canal in 1 case. 21 cases with surgical pathology results,including 16 cases diagnosed as phosphaturic mesenchymal tumor（ 1 case for low grade,the rest are benign）,5 cases for failing to do immunohistochemical studies only diagnosed as spindle cell tumor. 11 cases underwent CT scan,most lesions of uniform density,partly of a stippled calcification,2 lesions containing fat density,not all cases of hemorrhage,necrosis and cystic degeneration; CT value of-16-107 HU,with an average of 41. 0HU. Only one case enhanced and CT showed obvious homogeneous enhancement; bone and maxillofacial lesions with clear boundary and unclear soft tissue lesion boundary,juxtacortical bone lesions in 8 cases,which were seen in the seven patients with adjacent cortical bone destruction. 19 cases with MRI scan,the lesions on T1 are low signal,the soft tissue lesions in T2 were slightly high signal,next to the bone and cortical bone T2 lesions for high signal and the low signal or level mixed signal and gadolinium（ Gd-DTPA） enhancement showed obvious enhancement. Conclusion Because of the diversity of TIOtumor histopathology,the imaging findings are quite varied. Depending only on the imaging data for qualitative diagnosis is very difficult,but CT and MRI can be found to clearly show the lesions,to exclude other causes and considering that there is no family history of adult low phosphorus osteomalacia patients,CT and MRI of the abnormal imaging findings can make a prompt diagnosis,but also can be used for the diagnosis of TIO for preoperative evaluation and follow-up treatment.