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Solitary Cranial Langerhans Cell Histiocytosis: Two case reports
Insights
Langerhans cell histiocytosis (LCH) of the skull is a rare condition. This study details two cases of LCH in the orbit and skull, showing effective treatment outcomes.
Area of Science:
- Oncology
- Histopathology
- Immunohistochemistry
Background:
- Langerhans cell histiocytosis (LCH) is a clonal proliferative disorder of Langerhans cells.
- LCH can present as a unifocal or multifocal unisystem or multisystem disease.
- Orbital and skull involvement in LCH, particularly with a history of trauma, requires careful evaluation.
Observation:
- Two cases of unifocal erosive skull lesions with a history of trauma are presented.
- Histologic examination revealed numerous histiocytes, giant cells, and eosinophils.
- Immunohistochemistry confirmed the presence of Langerhans cells using CD1a and S100 markers.
Findings:
- LCH of the skull demonstrated characteristic histopathologic features.
- The described cases highlight the clinical spectrum of LCH in the orbit and skull.
- Both patients achieved positive outcomes with multimodal treatment, including surgical resection, steroids, and radiotherapy/chemotherapy.
Implications:
- Early diagnosis and appropriate treatment of LCH in the skull lead to excellent prognoses.
- Multidisciplinary management is crucial for optimizing outcomes in patients with LCH.
- Further research into the pathogenesis and treatment of LCH, especially in rare locations, is warranted.
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.