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

Skeletal Radiol. 2020 Dec;49(12):1925-1937. doi: 10.1007/s00256-020-03472-2. Epub 2020 May 26.

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 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.

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: Bone tumour; Langerhans cell histiocytosis; MRI, radiography.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Child, Preschool
  • Female
  • Histiocytosis, Langerhans-Cell* / diagnostic imaging
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Pelvis
  • Retrospective Studies
  • Shoulder*
  • Young Adult