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Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis |
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| Authors: | 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 |
| Affiliation: | 1.Division of Pediatric Oncology, Department of Pediatrics,University of S?o Paulo,S?o Paulo,Brazil;2.Department of Pathology,University of S?o Paulo,S?o Paulo,Brazil;3.Department of Genetics,University of S?o Paulo,S?o Paulo,Brazil;4.Radiology Division,University of S?o Paulo,S?o Paulo,Brazil;5.Division of Neurosurgery, Department of Surgery and Anatomy, Faculty of Medicine of Ribeir?o Preto,University of S?o Paulo,S?o Paulo,Brazil;6.Laboratório de Pediatria – Bloco G. Av. Bandeirantes,Hospital das Clínicas da Faculdade de Medicina de Ribeir?o Preto – USP,Ribeir?o Preto,Brazil |
| 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. |
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