Dion Elisabeth, Graef Claire, Miquel Anne, Haroche Julien, Wechsler Bertrand, Amoura Zahir, Zeitoun Delphine, Grenier Philippe A, Piette Jean-Claude, Laredo Jean-Denis
Department of Radiology, La Pitié Salpêtrière Hospital, 47-83 Boulevard de l'Hôpital, 75651 Paris Cedex 13, France.
Radiology. 2006 Feb;238(2):632-9. doi: 10.1148/radiol.2382041525. Epub 2005 Dec 21.
To retrospectively review the bone findings at radiography, scintigraphy, computed tomography (CT), and magnetic resonance (MR) imaging in 11 patients with immunohistochemical and histologic proof of Erdheim-Chester disease.
This study was designed as a retrospective review; approval of the institutional review board and patient consent were not required for this type of study. Eleven patients (eight men and three women; mean age, 49 years; range, 17-68 years) with Erdheim-Chester disease underwent conventional radiography of the skeleton and bone scintigraphy. Two patients underwent CT of the femora and 10 underwent CT of the skull. Eight patients underwent MR imaging. Conventional radiographs, bone scintigrams, CT scans, and MR images were reviewed in consensus by four musculoskeletal radiologists.
All 11 patients had involvement of the long bones and normal axial skeleton, hands, and feet. Bilateral and symmetric osteosclerosis of the diaphysis of the long bones was present in 52 (26 pairs) (98%) of the 53 bone lesions visible on conventional radiographs. Osteosclerosis was heterogeneous in 65% of the patients and homogeneous in 35%. Diaphysis was involved in 100% and metaphysis in 44 (83%) lesions. Partial epiphyseal involvement sparing the subchondral bone was present in 24 (45%) lesions. Periostitis was seen in 35 (66%) and endosteitis in 50 (94%) of the 53 long bones involved. Bone scintigraphy depicted tracer uptake in all bone lesions visible on radiographs. Skull and face bone lesions were present in two patients. MR imaging depicted a replacement of the normal fatty bone marrow by heterogeneous signal intensity on T1- and T2-weighted spin-echo images. Lesion extent, epiphyseal involvement, and periostitis were clearly depicted at MR imaging.
This series provides a detailed description of bone involvement in Erdheim-Chester disease. Periostitis and partial epiphyseal involvement of the long bones are also features of this disease. (c) RSNA, 2005.
回顾性分析11例经免疫组化和组织学证实的Erdheim-Chester病患者的X线摄影、骨闪烁显像、计算机断层扫描（CT）及磁共振成像（MR）的骨骼表现。
本研究为回顾性研究；此类研究无需获得机构审查委员会的批准及患者同意。11例Erdheim-Chester病患者（8例男性，3例女性；平均年龄49岁；年龄范围17 - 68岁）接受了骨骼的传统X线摄影及骨闪烁显像检查。2例患者进行了股骨CT检查，10例患者进行了颅骨CT检查。8例患者接受了MR成像检查。4位肌肉骨骼放射科医生共同对传统X线片、骨闪烁显像图、CT扫描及MR图像进行了评估。
所有11例患者均累及长骨，而中轴骨骼、手部及足部正常。在传统X线片上可见的53处骨病变中，52处（26对）（98%）出现双侧对称性长骨干骨皮质硬化。65%的患者骨皮质硬化不均匀，35%均匀。100%累及骨干，44处（83%）病变累及干骺端。24处（45%）病变出现部分骨骺受累，而软骨下骨未受累。在53处受累长骨中，35处（66%）可见骨膜反应，50处（94%）可见骨髓炎。骨闪烁显像显示在X线片上可见的所有骨病变均有放射性摄取。2例患者出现颅骨及面部骨病变。MR成像显示在T1加权和T2加权自旋回波图像上，正常脂肪骨髓被不均匀信号强度所取代。MR成像清晰显示了病变范围、骨骺受累情况及骨膜反应。
本系列研究详细描述了Erdheim-Chester病的骨骼受累情况。长骨的骨膜反应及部分骨骺受累也是本病的特征。（c）RSNA，2005年。
