Case of the Month: May, 2011, Page 5

Answer: D. Langerhans cell histiocytosis. Radiologic and pathologic findings are compatible with the diagnosis.

Additional CT images of this patient:

Discussion: Langerhans Cell Histiocytosis of the bone.

  • It is a rare expanding erosive histiocyte accumulation within medullary cavity
  • Cause not known; infectious agents (viral), immune system dysfunction, neoplastic mechanisms, genetic factors, cellular adhesion molecules proposed but not confirmed
  • Incidence 0.05-0.5/100,000 children/year
  • Most commonly affects children and adolescents
  • M:F 2:1
  • Represents < 1% of biopsy-proven primary bone lesions
  • Most common signs/symptoms:
    • Localized pain occurs secondary to medullary expansion or pathologic fracture
    • Fever, leukocytosis
    • Reduced mobility, swelling
  • Prognosis is variable depending on presentation age, extent of systemic disease. Spontaneous remission is common, usually beginning to regress after 3 months but may take years to completely resolve.
  • Treatment usually conservative with symptomatic relief and bracing. Occasionally surgical intervention (partial intralesional curettage, fusion), external beam radiotherapy, chemotherapy, or steroids is warranted in patients with neurological deficits or when conservative treatment fails.

References:

  • www.statdx.com
  • M Greenlee et al. Eosinophilic granuloma in the pediatric spine.PediatrNeurosurg. 2007;43(4):285-92.
  • JD Port. Langerhans' Cell Histiocytosis (Histiocytosis X) of Bone: A Clinicopathobogic Analysis of 263 Pediatric and Adult Cases. Radiology June 1996 199:724.
  • M A Stull, M J Kransdorf, and K O Devaney.Langerhans cell histiocytosis of bone. Radiographics July 1992 12:801-823.
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