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).
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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