Musculoskeletal imaging features of non-Langerhans cell histiocytoses

The non-Langerhans cell histiocytoses (N-LCH) represent a group of rare diseases with different clinical presentations and imaging features to classical LCH. While there is a long list of entities, only few present with musculoskeletal soft tissue and osseous manifestations alongside the more commonly reported systemic findings. Erdheim-Chester disease (ECD) is typically seen in adults as bilateral and symmetrical long bone osteosclerosis. Rosai-Dorfman disease (RDD) is more commonly seen in children and young adults with bone involvement usually being a manifestation of extra-nodal disease. Primary osseous RDD is very rare, with both displaying rather non-specific imaging features of an expansile lucent lesion with or without an extra-osseous component. Juvenile xanthogranuloma (JXG) is a benign disorder typically seen in very young children. The most common imaging manifestation is a dermal or sub-dermal soft tissue mass. This article reviews the musculoskeletal imaging appearances of the commoner N-LCH.