Langerhans cell histiocytosis of the shoulder girdle, pelvis and extremities: a review of radiographic and MRI features
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SCIENTIFIC ARTICLE
Langerhans cell histiocytosis of the shoulder girdle, pelvis and extremities: a review of radiographic and MRI features in 85 cases J Singh 1 & R Rajakulasingam 2
&
A Saifuddin 2
Received: 7 April 2020 / Revised: 13 May 2020 / Accepted: 14 May 2020 # ISS 2020
Abstract Objectives To describe the radiographic and MRI features of histologically proven Langerhans cell histiocytosis (LCH) of the bone. Materials and methods A retrospective review of the radiographic and MRI features of 85 histologically proven cases of skeletal LCH over a 12-year period. Clinical data recorded included age, gender and location. Radiographic features evaluated included Lodwick grading, cortical/periosteal response and matrix mineralisation. MRI features assessed included lesion size and T1weighted signal intensity (T1W SI), nature of margin, hypointense rim, enhancement pattern, bone marrow and soft tissue oedema, soft tissue mass, fluid-fluid levels, the penumbra sign and the budding and bulging signs. Results The study included 85 patients, 54 males and 31 females with mean age of 13 years (range 1–76 years). The femur was the commonest bone involved (38.8%), followed by the scapula (9.4%), clavicle (8.2%), ilium (8.2%) and ischium (8.2%). The mean maximal lesion size was 40 mm (range 16–85 mm). The commonest radiographic appearance was of a lytic lesion with no appreciable sclerotic rim, an intact expanded cortex and either absent or laminated periosteal response. MRI demonstrated a hypointense rim (41.5%), the budding (31.7%) and bulging (36.6%) signs, eccentric extra-osseous mass (42.7%), prominent bone marrow (95.3%) and soft tissue oedema (84.1%). Rarer features included haemorrhage (2.4%), the penumbra sign (3.5%) and fluid-fluid levels (2.4%). Thirteen of 25 post-contrast studies showed peripheral/rim enhancement with central necrosis. Conclusions LCH classically presents as a moderately aggressive lytic bone lesion on radiography, with prominent reactive bone and soft tissue oedema being a characteristic feature on MRI. Keywords Langerhans cell histiocytosis . MRI, radiography . Bone tumour
Introduction Langerhans cell histiocytosis (LCH) is a multi-system disorder characterised by abnormal proliferation of histiocytic cells
* R Rajakulasingam [email protected] J Singh [email protected] A Saifuddin [email protected] 1
Department of Medical Imaging, Guru Nanak Dev Superspeciality hospital, Goindwal Sahib Road, Taran Sahib, Punjab 143401, India
2
Department of Medical Imaging, Royal National Orthopaedic Hospital, Stanmore, Middlesex HA7 4LP, UK
which arise from the monocyte-macrophage cell lineage [1]. Although the exact pathology of this disease is unknown, recent findings of an extracellular signal-regulated kinases pathway mutation lead to its classification as a myeloid neoplasm [2, 3]. The disease can involve any body system but most commonly affects the bone marrow, liver, spleen and lymph nodes. The localised form was previously referred to as eosinophilic granuloma,
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