Erdheim-Chester病骨质受累的影像学表现及临床分析

Image Andclinicalanalysis of Bone Involvement in Erdheim-Chester Disease

目的分析Erdheim-Chester病(ECD)的临床及影像特点,以提高诊断水平。方法回顾性分析经病理证实的8例Erdheim-Chester病患者的临床及影像资料,其中X线平片检查5例,CT检查7例,MRI检查4例(增强1例),SPECT检查3例。结果8例均累及骨骼,2例患者有骨骼外症状,包括发热、头晕乏力、突眼、下肢水肿、多饮多尿、间质性肺炎等。8例骨骼病变均累及下肢,5例表现为多发对称性病变,其中4例表现为双下肢长骨对称性骨质硬化,1例表现为长骨对称性膨胀性骨质破坏,2例行MRI检查,病变呈T1WI低信号、T2WI高信号,3例行SPECT检查,表现为病变部位放射性核素浓聚;3例表现为单发假瘤样病变,呈溶骨性骨质破坏,2例行MRI检查,病变呈T1WI低信号、T2WI高信号、高低混杂信号,1例增强扫描不均匀强化。结论ECD骨质受累主要表现为多发对称性病变,即双下肢对称性、弥漫性骨质硬化,也可见单发假瘤样病变,后者预后更好,最终确诊仍依赖病理。

Objective To investgate the clinical and imaging characteristics of Erdheim-Chester’s disease(ECD)in order to improve the clinical diagnosis.Methods Data and images of 8 patients with pathologically confirmed diagnosis as erdheim-Chester’s disease from January 2010 to January 2019,were retrospectively analyzed.These included 4 males and 4 females,age ranging from 42-55 years and median age was 50 years.The clinical and imaging data were collected,including 5 cases of X-ray,7 cases of CT,4 cases of MR(1 patients underwent enhanced scan)and 3 cases of SPECT.Results All 8 patients had involvement of the skeletal system,2 patients suffered other symptoms,including fever,dizziness and weakness,exophthalmos,lower extremity edema,diabetes insipidus and interstitial lung disease.All 8 cases of skeletal lesions were located in the lower extremities.4 cases showed symmetric sclerosis of the long bones,and 1 cases showed symmetric expansile bone destruction,2 of which presented with low signal intensity on T1WI,high signal intensity on T2WI,and 3 of which presented radionuclide uptake at the lesion site on SPECT images.3 cases showed focal pseudotumoral lesions and exhibited osteolysis,2 of which presented with low signal intensity on T1WI,high or heterogeneous signal intensity on T2WI,and 1 presented with inhomogeneous enhancement.Conclusion There are two distinct patterns of bone involvement in ECD,one is the typical diffuse skeletal involvement,with symmetric sclerosis of the long bones and the other is focal pseudotumoral lesions,which have a better prognosis.The final diagnosis still depends on pathology.