Solitary Cranial Langerhans Cell Histiocytosis: Two case reports

Hiroshima J Med Sci. 2015 Dec;64(4):59-63.

Abstract

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as a unisystem (unifocal or multifocal) or multisystem disease. We describe the clinical and histologic spectrum of LCH of the orbit and skull in our two cases. Both cases had unifocal erosive skull lesions with a history of trauma. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. LCH has an excellent prognosis when treated with surgical resection, steroids and radiotherapy or chemotherapy. One of our patients is disease free at 7 year follow-up and one patient had regression of lesion on follow-up.

Publication types

  • Case Reports

MeSH terms

  • Antigens, CD1 / analysis
  • Biomarkers / analysis
  • Biopsy
  • Child
  • Child, Preschool
  • Craniocerebral Trauma / immunology
  • Craniocerebral Trauma / pathology*
  • Craniocerebral Trauma / therapy
  • Histiocytosis, Langerhans-Cell / immunology
  • Histiocytosis, Langerhans-Cell / pathology*
  • Histiocytosis, Langerhans-Cell / therapy
  • Humans
  • Immunohistochemistry
  • Langerhans Cells / immunology
  • Langerhans Cells / pathology*
  • Magnetic Resonance Imaging
  • Male
  • Orbital Diseases / immunology
  • Orbital Diseases / pathology*
  • Orbital Diseases / therapy
  • Remission Induction
  • S100 Proteins / analysis
  • Skull / immunology
  • Skull / pathology*
  • Time Factors
  • Tomography, X-Ray Computed
  • Treatment Outcome

Substances

  • Antigens, CD1
  • Biomarkers
  • CD1a antigen
  • S100 Proteins