title: Are patients with encephalocraniocutaneous lipomatosis at increased risk of developing low-grade gliomas?
abstract: BACKGROUND
  Cancer-prone genetic disorders are responsible for brain tumors in a considerable proportion of children. Additionally, rare genetic syndromes associated to cancer development may potentially disclose genetic mechanisms related to oncogenesis.
  
  
  METHODS
  We describe two pediatric patients with encephalocraniocutaneous lipomatosis (ECCL), a very rare genetic syndrome with around 60 reported cases, which developed low-grade astrocytoma at 3 and 12 years of age.
  
  
  RESULTS
  Patients with ECCL seem to be at risk of benign forms of osseous tumors such as ossifying fibromas, odontomas, and osteomas.
  
  
  CONCLUSIONS
  The association between brain tumor and ECCL was previously reported only once, in a pediatric case of a mixed neuronal-glial histology. Whether ECCL may be a genetic condition of predisposing brain tumor in children strongly needs to be addressed.
authors: Department of Pediatrics, School of Medicine of Ribeirão Preto, University of São Paulo, Ribeirão Preto, Brazil. etvalera@hcrp.fmrp.usp.br
authors: Elvis Terci
authors: ET
authors: Valera
authors: María Sol
authors: MS
authors: Brassesco
authors: Carlos Alberto
authors: CA
authors: Scrideli
authors: Marcus Vinicius
authors: MV
authors: de Castro Barros
authors: Antonio Carlos
authors: AC
authors: Santos
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* / Q000473:pathology; D001932:Brain Neoplasms / Q000150:complications* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005128:Eye Diseases / Q000150:complications* / Q000473:pathology; D006801:Humans; D008068:Lipomatosis / Q000150:complications* / Q000473:pathology; D060787:Neoplasm Grading; D020752:Neurocutaneous Syndromes / Q000150:complications* / Q000473:pathology; D012307:Risk Factors