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Case Reports

Abstract

Juvenile xanthogranuloma (JXG) is an inflammatory histiocytic disorder that is typically distinguished from other histiocytic conditions by its characteristic histologic features of foamy histiocytes and Touton giant cells.  However, early or non-lipidized form of JXG may lack these definitive findings, making diagnosis more challenging. Immunophenotypic characterization is occasionally employed in these cases to elucidate the diagnosis and delineate early JXGs from their histologic mimickers, including atypical fibroxanthoma, leiomyosarcoma, myelogenous leukemia cutis, and myeloid sarcoma. Immunohistochemistry (IHC) studies can result in erroneous diagnosis that may have serious clinical implications, especially in the absence of clinical correlation. We present the case of a patient with an early JXG which was initially misdiagnosed as myeloid sarcoma and discuss the importance of clinicopathologic correlation and cautious interpretation of ancillary studies.