Brain Tumors Research Today is a free monthly online journal that collates and summarizes the latest research about Brain Tumors, including details on symptoms, benign and malignant tumors, gliomas, screening, treatment.

Central nervous system juvenile xanthogranuloma with malignant transformation.

Orsey A, Paessler M, Lange BJ, Nichols KE

Division of Pediatric Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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.

Published 25 February 2008 in Pediatr Blood Cancer, 50(4): 927-30.
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