Erschienen in:
01.02.2010 | Case Report
Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis
verfasst von:
María Sol Brassesco, Elvis Terci Valera, Aline Paixão Becker, Angel Mauricio Castro-Gamero, André de Aboim Machado, Antônio Carlos Santos, Carlos Alberto Scrideli, Ricardo Santos Oliveira, Hélio Rubens Machado, Luiz Gonzaga Tone
Erschienen in:
Journal of Neuro-Oncology
|
Ausgabe 3/2010
Einloggen, um Zugang zu erhalten
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.