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Shows University, School of Medicine Tokyo 142-8666, Japan
Erdheim-Chester disease is a distinctive radiologic and pathologic entity involving many organs including long bones, neurogenic organs, muscle, breast, and others and differs from other histiocytic disorders, particularly Langerhans' cell histiocytosis. Only three published cases describing MR imaging findings of Erdheim-Chester disease in long bones have been reported in English literature of which one case was probable [1,2,3].
A 68-year-old woman was admitted for evaluation with a 2-year history of bilateral lower extremity edema. A radiograph of both lower extremities showed lytic lesions superimposed on internal sclerotic architectures symmetrically in both distal and proximal metaphyses of the femur (Fig. 2A). A coronal T1-weighted spin-echo MR image (TR/TE, 520/25) showed diffusely invasive bone marrow masses destroying cortical bones in both tibias (Fig. 2B). The signal intensity of these masses was the same as that of muscle, but areas of low signal intensity appeared in the masses. A sagittal gadolinium-enhanced MR image of the left lower extremity showed that the bone marrow masses were not enhanced homogeneously (Fig. 2C), and the cystic component was nonenhancing. An axial T2-weighted MR image (2000/80) of both lower extremities showed bone marrow masses with higher signal intensity than that of muscle (Fig. 2D). Cystic areas were also noted within these masses. Internal sclerotic architectures seen on a radiograph appeared to be low-signal-intensity areas both on T1- and T2-weighted MR images. Partial resection of extraosseous masses was performed, and a histopathologic diagnosis of Erdheim-Chester disease was confirmed.
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Radiographic features of Erdheim-Chester disease involving long bones are bilateral symmetric sclerosis of metaphyseal and diaphyseal regions of the long bones with epiphyseal sparing [2,3,4]. In addition to the classic sclerotic changes, 30-40% of the patients also have some lytic component [2]. A few features of the MR findings have been reported [1,2,3]. T1-weighted MR images have shown extensive replacement of the healthy fatty marrow with low-signal-intensity soft-tissue masses. Nonhomogeneous high signal intensity has been seen on T2-weighted sequences with a mass protruding into the surrounding tissues. In our patient, a cystic component and low-signal-intensity areas were noted on both T1- and T2-weighted MR images. Differential diagnostic considerations include adamantinoma of the tibia, fibrous dysplasia, chronic osteomyelitis, tuberculous and fungal osteomyelitis bone infarction, and Engelmann's disease. However, bilateral and symmetric replacement of the healthy fatty marrow with cortical destruction and MR findings of an associated soft-tissue mass protruding into the surrounding tissues may be diagnostic of Erdheim-Chester disease.
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  E. Dion, C. Graef, A. Miquel, J. Haroche, B. Wechsler, Z. Amoura, D. Zeitoun, P. A. Grenier, J.-C. Piette, and J.-D. Laredo Bone Involvement in Erdheim-Chester Disease: Imaging Findings including Periostitis and Partial Epiphyseal Involvement Radiology, December 21, 2005; (2005) 2382041525. [Abstract] [Full Text]
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