Atypical skeletal involvement in patients with Erdheim–Chester disease: CT imaging findings

Abstract

Abstract Objectives To review retrospectively atypical bone findings from computed tomographic (CT) imaging in patients with Erdheim–Chester disease. Methods All 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. Results Twenty-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. Conclusions This 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.