Abstract
ObjectivesTo review retrospectively atypical bone findings from computed tomographic (CT) imaging in patients with Erdheim–Chester disease.MethodsAll 28 patients with Erdheim–Chester disease (13 men and 15 women; mean age, 45 years; range, 7–63 years) underwent chest-abdomen-pelvis CT. CT images were reviewed and analyzed for the various features of atypical bone lesions by two radiologists in consensus.ResultsTwenty-one patients had atypical bone involvement. Radiologically, these atypical osseous lesions were categorized into three types: diffuse, nodular and patchy. Eleven (52%) of the 21 patients had spinal lesions, of which four (36%) had the diffuse type, eight (73%) had the nodular pattern, and six (55%) had the patchy pattern. Sixteen (76%) of the 21 patients had pelvic involvement, of which two (13%) were diffuse, nine (56%) were nodular and 11 were (69%) patchy. Ribs were involved in seven (33%) of the 21 patients, with the nodular pattern in one (14%) patient and the patchy type in six (86%) patients. Clavicle involvement was seen in nine (43%) of the 21 patients, of which the diffuse type was found in only one (11%) patient, the nodular type in six (67%) patients, the solitary patchy type in four (44%) patients. Sternum involvement was seen in 10 (48%) of the 21 patients and all were nodular.ConclusionsThis series provides a detailed description of atypical bone involvement in Erdheim–Chester disease which on CT displays three major patterns. Understanding these patterns may help increase the accuracy of diagnosis of this disease.
Highlights
Erdheim–Chester disease (ECD) is a rare, non-Langerhans histiocytosis characterized by the infiltration of tissues by foamy CD68+CD1a− histiocytes [1,2,3]
Given that the clinical and radiologic presentations of extraosseous involvement in ECD are diverse and nonspecific, the distinctive imaging findings of its skeletal involvement provide an important clue to the accurate diagnosis of this condition
We report the computed tomography (CT) imaging findings of skeletal involvement in a series of 28 patients with ECD, with emphasis on some aspects that, to our knowledge, have not been systematically described before: radiological features of atypical bone involvement in the spine, pelvis, ribs, clavicles, and sternum
Summary
Erdheim–Chester disease (ECD) is a rare, non-Langerhans histiocytosis characterized by the infiltration of tissues by foamy CD68+CD1a− histiocytes [1,2,3] It is a systemic and neoplastic disorder, first described by Jakob Erdheim and William Chester in 1930 [4]. The typical imaging findings of long tubular bones in ECD have been systemically described [6], rare and atypical skeletal involvement, including spine, pelvis (ilium, ischium and pubis), ribs, clavicles, and sternum, has been described generally in case reports or mentioned lightly in small series [5, 8,9,10,11,12,13,14,15,16,17]. Our study aimed to describe atypical skeletal involvement (spine, pelvis, ribs, clavicles, and sternum) in ECD by retrospectively reviewing CT images of the chest-abdomen-pelvis with abnormal findings in 28 patients with ECD
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