Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis.
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.
Brassesco
María Sol
MS
Division of Pediatric Oncology, Department of Pediatrics, University of São Paulo, São Paulo, Brazil. marsol@rge.fmrp.usp.br
Valera
Elvis Terci
ET
Becker
Aline Paixão
AP
Castro-Gamero
Angel Mauricio
AM
de Aboim Machado
André
A
Santos
Antônio Carlos
AC
Scrideli
Carlos Alberto
CA
Oliveira
Ricardo Santos
RS
Machado
Hélio Rubens
HR
Tone
Luiz Gonzaga
LG
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*