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(Reference retrieved automatically from Web of Science through information on FAPESP grant and its corresponding number as mentioned in the publication by the authors.)

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

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Valera, Elvis Terci [1, 2] ; Brassesco, Maria Sol [1] ; Scrideli, Carlos Alberto [1] ; de Castro Barros, Marcus Vinicius [3] ; Santos, Antonio Carlos [4] ; Oliveira, Ricardo Santos [5] ; Machado, Helio Rubens [5] ; Tone, Luiz Gonzaga [1]
Total Authors: 8
[1] Univ Sao Paulo, Dept Pediat, Sch Med Ribeirao Preto, BR-14049 Ribeirao Preto - Brazil
[2] Ribeirao Preto USP, Dept Pediat, Hosp Clin, Fac Med, Ribeirao Preto, SP - Brazil
[3] Univ Sao Paulo, Dept Pathol, Sch Med Ribeirao Preto, BR-14049 Ribeirao Preto - Brazil
[4] Univ Sao Paulo, Div Radiol, Sch Med Ribeirao Preto, BR-14049 Ribeirao Preto - Brazil
[5] Univ Sao Paulo, Div Neurosurg, Sch Med Ribeirao Preto, BR-14049 Ribeirao Preto - Brazil
Total Affiliations: 5
Document type: Journal article
Source: CHILD'S NERVOUS SYSTEM; v. 28, n. 1, p. 19-22, JAN 2012.
Web of Science Citations: 11

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. (AU)

FAPESP's process: 10/15717-0 - Analysis of chromosomal aberrations in childhood cancer through conventional and molecular cytogenetics
Grantee:Elvis Terci Valera
Support type: Regular Research Grants
FAPESP's process: 10/16652-9 - NF-kB inhibition in glioblastoma: in vitro and in vivo effects of DHMEQ in tumor chemoresistance, invasion and progression
Grantee:María Sol Brassesco Annichini
Support type: Scholarships in Brazil - Young Researchers