Langerhans cell histiocytosis of the shoulder girdle, pelvis and extremities: a review of radiographic and MRI features in 85 cases.

objectivesTo describe the radiographic and MRI features of histologically proven Langerhans cell histiocytosis (LCH) of the bone. MATERIALS AND

methodsA 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 T1-weighted 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.

resultsThe 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.

conclusionsLCH 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.