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Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis

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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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Acknowledgement

This research was supported by Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP Proc. No: 07/54236-4, 06/04827-3 and 07/04065-9).

Author information

Authors and Affiliations

  1. Division of Pediatric Oncology, Department of Pediatrics, University of São Paulo, São Paulo, Brazil

    María Sol Brassesco, Elvis Terci Valera, Carlos Alberto Scrideli & Luiz Gonzaga Tone

  2. Department of Pathology, University of São Paulo, São Paulo, Brazil

    Aline Paixão Becker

  3. Department of Genetics, University of São Paulo, São Paulo, Brazil

    Angel Mauricio Castro-Gamero

  4. Radiology Division, University of São Paulo, São Paulo, Brazil

    André de Aboim Machado & Antônio Carlos Santos

  5. Division of Neurosurgery, Department of Surgery and Anatomy, Faculty of Medicine of Ribeirão Preto, University of São Paulo, São Paulo, Brazil

    Ricardo Santos Oliveira & Hélio Rubens Machado

  6. Laboratório de Pediatria – Bloco G. Av. Bandeirantes, Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto – USP, 3900. Bairro Monte Alegre, CEP 14048-900, Ribeirão Preto, SP, Brazil

    María Sol Brassesco

Authors
  1. María Sol Brassesco
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  2. Elvis Terci Valera
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  3. Aline Paixão Becker
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  4. Angel Mauricio Castro-Gamero
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  5. André de Aboim Machado
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  6. Antônio Carlos Santos
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  7. Carlos Alberto Scrideli
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  8. Ricardo Santos Oliveira
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  9. Hélio Rubens Machado
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  10. Luiz Gonzaga Tone
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Corresponding author

Correspondence to María Sol Brassesco.

Additional information

María Sol Brassesco and Elvis Terci Valera have contributed equally.

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Brassesco, M.S., Valera, E.T., Becker, A.P. et al. Low-grade astrocytoma in a child with encephalocraniocutaneous lipomatosis. J Neurooncol 96, 437–441 (2010). https://doi.org/10.1007/s11060-009-9978-1

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  • DOI: https://doi.org/10.1007/s11060-009-9978-1

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