Skeletal staging in Langerhans cell histiocytosis. A multimodality imaging review

Evaluating the extent of skeletal disease in Langerhans cell histiocytosis (LCH) is a major predictor of patient outcome. Traditionally, whole-body skeletal staging consists of plain radiography and bone scintigraphy. However, more recently whole-body MRI has been shown to be accurate in detecting osseous and extra-osseous lesions, but no large-scale studies analysing its role within the diagnostic algorithm of LCH skeletal staging currently exist. In addition, FDG PET-CT provides useful information regarding disease activity and treatment response, but has an inherent radiation dose which is not ideal in children. Currently, radiographic skeletal survey remains the gold standard with cross-sectional imaging only performed for further characterisation. However, radiographs have shown a wide sensitivity range for skeletal staging and have clear limitations in detecting extra-skeletal disease, a crucial component of stratification in identifying 'at risk' organs. We aim to highlight the various appearances of bony LCH across all the imaging modalities for primary skeletal staging. We will also review the advantages, disadvantages, sensitivity and specificity of each, and establish their role in staging skeletal LCH. Recent studies using whole-body MRI have shown promising results, with radiographs and other modalities playing a more complementary role.