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Published on: May 10, 2017
Xanthogranulomatous Epithelial Tumor in a Pediatric Patient. A Potential Diagnostic Pitfall With Non-Langerhans Cell
Danielle V de Stefano1, Jennifer Picarsic2,3, Somak Roy4,5
1Department of Pathology and Laboratory Medicine, Phoenix Children's Hospital, Phoenix, AZ.
Abstract:
Xanthogranulomatous epithelial tumor is a recently described neoplasm harboring recurrent HMGA2::NCOR2 fusions, which, owing to its extensive xanthogranulomatous appearance with abundant foamy histiocytes and frequent Touton-type giant cells, may be confused with other inflammatory and neoplastic entities. We present a case arising in the right arm subcutaneous tissues of a 10-year-old boy, which was initially interpreted as a non-Langerhans cell histiocytic neoplasm compatible with Juvenile xanthogranuloma.

