Langerhans Cell Histiocytosis

  • Etiology: Abnormal proliferation of Langerhans cells
  • Imaging: Skeletal survey to assess for and monitor distant lesions
  • Imaging Radiograph Skull: Lucent lesion without sclerotic margin with beveled edges with beveled edge appearance due to skull lesion involving outer table more than inner table
  • Imaging Radiograph Spine: Vertebra plana with soft tissue paravertebral mass
  • Imaging Radiograph:
    — Distribution in flat bones and long bones
    — Well circumscribed lytic lesions with with or without periosteal reaction and with or without sclerotic rim
    — Floating teeth,
  • Imaging MRI:
    — Lesions filled with soft tissue without large mas
    — Often homogeneously bright and enhancing
    — With or without marrow edema much more than soft tissue edema
  • DDX:
  • Complications:
  • Treatment:
  • Clinical:
    — Common presentation is lucent skull lesion
    — Most common cause of vertebra plana in children

Radiology Cases of Langerhans Cell Histiocytosis

Radiograph of Langerhans Cell Histiocytosis of the femur
AP radiograph of the pelvis shows a well circumscribed lytic lesion in the proximal right femoral metaphysis without any associated periosteal reaction.

Radiology Cases of Langerhans Cell Histiocytosis Causing Vertebra Plana

Radiograph and MRI of Langerhans cell histiocytosis of the spine
Lateral radiograph of the lumbar spine shows loss of height of the L1 vertebral body. Coronal and sagittal T1 MRI with contrast of the lumbar spine shows flattening (vertebra plana) and enhancement of the L1 vertebral body with a small enhancing right sided paravertebral mass.
CT and MR of vertebra plan in Langerhans cell histiocytosis
Sagittal CT without contrast of the spine (far left) shows a marked compression deformity of the T7 vertebral body which is almost flat in appearance. Sagittal T2 MRI of the spine (near left) shows again loss of height in the T7 vertebral body with the T6-T7 and T7-T8 intervertebral disks in close approximation to each other. There is evidence of a high signal intensity soft tissue mass anterior and posterior to the T7 vertebral body. Sagital T1 MRI of the spine without (near right) and with contrast (far right) show enhancement of this soft tissue mass anterior and posterior to T7 which has an extradural component which is compressing the spinal cord at this level.