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Central nervous system juvenile xanthogranuloma with malignant transformation
Author(s) -
Orsey Andrea,
Paessler Michele,
Lange Beverly J.,
Nichols Kim E.
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21252
Subject(s) - juvenile xanthogranuloma , medicine , histiocyte , pathology , central nervous system , pathological , bone marrow , biopsy , neoplasm , disease
Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder that typically manifests in the skin. Here, we describe a patient with JXG diffusely involving the central nervous system (CNS), whose disease responded to therapy but subsequently underwent dissemination to the peritoneum and bone marrow. Repeat biopsy at dissemination revealed pleomorphic histiocytes with tetraploidy, suggesting evolution to a clonal histiocytic neoplasm. Despite further chemotherapy, the patient died of disease progression. This case highlights the clinical and pathological heterogeneity of JXG and the difficulty of treating multi‐focal CNS disease. Pediatr Blood Cancer 2008;50:927–930. © 2007 Wiley‐Liss, Inc.