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    J Neurooncol. 2010 Feb;96(3):437-41. Epub 2009 Aug 4.

    Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis.

    Source

    Division of Pediatric Oncology, Department of Pediatrics, University of São Paulo, São Paulo, Brazil. marsol@rge.fmrp.usp.br

    Abstract

    Encephalocutaneous lipomatosis (ECCL), or Haberland syndrome, is an uncommon congenital disorder with unique cutaneous, ocular and neurological features. In the present article, we describe a 3-year-old boy with ECCL who developed an extensive and recurring intraventricular low-grade glioma with atypical pathological features and elevated mitotic index. Cytogenetic analysis from tumor sample was also performed. This is the first report of a low-grade astrocytoma occurring in a child with ECCL. Whether or not the origin of the tumor is associated to the pathogenesis of the underlying syndrome is a matter for further investigation.

    PMID:
    19652916
    [PubMed - indexed for MEDLINE]

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