Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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May 4, 2015
Langerhans Cell Histiocytosis (LCH)
- Poorly understood pathology with histiocytic proliferation and infiltration.
- Most common in children between 6 and 10 years of age. Male predilection. Localized pain is a common complaint.
- Key Diagnostic Features: Well-defined, sharply marginated, destructive lesions involving any portion of the cranial vault. CT demonstrates the osseous component and MRI demonstrates any associated soft tissue extension. It is important to consider the possibility of LCH in pediatric patients with destructive cranial vault lesions. These patients respond to chemotherapy, and surgical resection is, therefore, not necessary. In fact, reconstitution of bone can be seen in some treated cases.
- DDx:
- Metastasis
- Ewing sarcoma
- Rhabdomyosaroma
- Lymphoma
- Aggressive infective process
- Rx: Chemotherapy with radiation therapy. Surgical resection, only if absolutely necessary.
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