title: Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis.
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.
authors: Division of Pediatric Oncology, Department of Pediatrics, University of São Paulo, São Paulo, Brazil. marsol@rge.fmrp.usp.br
authors: María Sol
authors: MS
authors: Brassesco
authors: Elvis Terci
authors: ET
authors: Valera
authors: Aline Paixão
authors: AP
authors: Becker
authors: Angel Mauricio
authors: AM
authors: Castro-Gamero
authors: André
authors: A
authors: de Aboim Machado
authors: Antônio Carlos
authors: AC
authors: Santos
authors: Carlos Alberto
authors: CA
authors: Scrideli
authors: Ricardo Santos
authors: RS
authors: Oliveira
authors: Hélio Rubens
authors: HR
authors: Machado
authors: Luiz Gonzaga
authors: LG
authors: Tone
mesh_terms: D001254:Astrocytoma / Q000150:complications*; D001932:Brain Neoplasms / Q000150:complications*; D006801:Humans; D007223:Infant; D008068:Lipomatosis / Q000150:complications*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D020752:Neurocutaneous Syndromes / Q000150:complications*