Skeletal staging in Langerhans cell histiocytosis: a multimodality imaging review
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REVIEW ARTICLE
Skeletal staging in Langerhans cell histiocytosis: a multimodality imaging review Ramanan Rajakulasingam 1
&
Mateen Siddiqui 2 & Maria Michelagnoli 3 & Asif Saifuddin 1
Received: 20 September 2020 / Revised: 29 October 2020 / Accepted: 8 November 2020 # ISS 2020
Abstract 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. Keywords Langerhans cell histiocytosis . Radiographic skeletal survey . Whole-body bone scan . Whole-body MRI . FDG PET-CT
Introduction
* Ramanan Rajakulasingam [email protected] Mateen Siddiqui [email protected] Maria Michelagnoli [email protected] Asif Saifuddin [email protected] 1
Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore HA7 4LP, UK
2
Department of Radiology, North West Anglia Foundation Trust, Hinchingbrooke Hospital, Huntingdon, Cambridgeshire PE29 6NT, UK
3
Department of Paediatric Oncology, University College Hospital, Bloomsbury, London NW1 2BU, UK
According to the 2020 WHO Classification of Soft Tissue and Bone Tumours, Langerhans cell histiocytosis (LCH) represents a ‘clonal neoplastic proliferation of myeloid dendritic cells expressing a Langerhans cell phenotype’ [1]. The exact pathogenesis is still unknown, with an ongoing debate as to whether LCH is a reactive or neoplastic process characterised by accumulation of mononuclear phagocytes and granulomatous lesions. The latter is more widely accepted, with the term ‘inflammatory myeloid neoplasia’, accurately encompassing the reactive and neoplastic components of LCH [2–5]. LCH may affect any age group, but the majority are children less than 15 years of age. It can involve many organ systems, but most commonly the lungs, skin and skele
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