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.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)USP, Pediat Lab, Hosp Clin, Fac Med Ribeirao Preto, BR-14048900 Ribeirao Preto, SP, BrazilUniv São Paulo, Div Pediat Oncol, Dept Pediat, São Paulo, BrazilUniv São Paulo, Dept Pathol, São Paulo, BrazilUniv São Paulo, Dept Genet, São Paulo, BrazilUniv São Paulo, Div Radiol, São Paulo, BrazilUniv São Paulo, Div Neurosurg, Dept Surg & Anat, Fac Med Ribeirao Preto, São Paulo, BrazilFAPESP: 07/54236-4FAPESP: 06/04827-3FAPESP: 07/04065-9Web of Scienc