Are patients with encephalocraniocutaneous lipomatosis at increased risk of developing low-grade gliomas?

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2012-01-01
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Valera, Elvis Terci
Brassesco, Maria Sol
Scrideli, Carlos Alberto
Castro Barros, Marcus Vinicius de
Santos, Antonio Carlos
Oliveira, Ricardo Santos
Machado, Helio Rubens
Tone, Luiz Gonzaga
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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.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.Patients with ECCL seem to be at risk of benign forms of osseous tumors such as ossifying fibromas, odontomas, and osteomas.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.
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Childs Nervous System. New York: Springer, v. 28, n. 1, p. 19-22, 2012.