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Eosinophilic granuloma of bone in Adult
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Eosinophilic granuloma of bone in Adult

Contributors: Amy Phan BS, Benjamin L. Mazer MD, MBA, David Sullo MD, Eric Ingerowski MD, FAAP
Other Resources UpToDate PubMed

Synopsis

Langerhans cell histiocytosis (LCH) refers to a spectrum of diseases that includes eosinophilic granuloma (EG), Hand-Schuller-Christian disease, and Letterer-Siwe disease. EG is a rare (seen in 1-5 per million), benign tumor-like disorder that features clonal proliferation of Langerhans cells (antigen-presenting mononuclear cells of dendritic origin) usually found in bones; however, this can involve other organ systems. EG occurs most often in children aged 5-15 years, with a male-to-female ratio of 2:1. EG constitutes 60%-80% of cases of LCH and accounts for less than 1% of all bone tumors. Ninety percent of cases involve a single lesion, usually in the axial skeleton, with just under 10% of cases involving lesions in multiple bones or lesions in other organ systems (ie, skin, pituitary gland, gastrointestinal [GI] tract, lung, spleen, or brain).

The clinical presentation of EG depends largely on the location affected. EG is most often confined to the axial skeleton. EGs are often asymptomatic and found incidentally, or may initially present with pain and swelling of the affected area, with or without decreased range of motion. Commonly affected areas of the skeleton include (in order of frequency): skull, femur, pelvis, mandible, clavicle, ribs, and long bones (of the diaphysis and metaphysis).

Within the spine, EG accounts for 6.5%-25% of all spinal tumors, with the most common location being the thoracic spine, followed by the lumbar and then cervical spine. In cases of EG of the spine, the following symptoms have been reported: neck / back pain, limb weakness, neck / back stiffness with restriction of movements, kyphotic deformity, radiculopathy, and torticollis.

Codes

ICD10CM:
C96.6 – Unifocal Langerhans-cell histiocytosis

SNOMEDCT:
238476000 – Eosinophilic granuloma of bone

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Last Reviewed:03/18/2024
Last Updated:04/04/2024
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Eosinophilic granuloma of bone in Adult
Imaging Studies image of Eosinophilic granuloma of bone - imageId=8322100. Click to open in gallery.  caption: '<span>Axial CT of the pelvis  demonstrates a large lytic lesion involving the right hip with cortical  destruction. This pediatric patient also had multiple other sites of  lytic lesions, including the skull. Given the constellation of findings,  this is most compatible with an eosinophilic granuloma.</span>'
Axial CT of the pelvis demonstrates a large lytic lesion involving the right hip with cortical destruction. This pediatric patient also had multiple other sites of lytic lesions, including the skull. Given the constellation of findings, this is most compatible with an eosinophilic granuloma.
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