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01-01-2012 | Brief Communication

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

Authors: Elvis Terci Valera, María Sol Brassesco, Carlos Alberto Scrideli, Marcus Vinicius de Castro Barros, Antonio Carlos Santos, Ricardo Santos Oliveira, Hélio Rubens Machado, Luiz Gonzaga Tone

Published in: Child's Nervous System | Issue 1/2012

Abstract

Introduction

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.

Method

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.

Results

Patients with ECCL seem to be at risk of benign forms of osseous tumors such as ossifying fibromas, odontomas, and osteomas.

Conclusion

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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Title: Are patients with encephalocraniocutaneous lipomatosis at increased risk of developing low-grade gliomas?
Authors: Elvis Terci Valera
María Sol Brassesco
Carlos Alberto Scrideli
Marcus Vinicius de Castro Barros
Antonio Carlos Santos
Ricardo Santos Oliveira
Hélio Rubens Machado
Luiz Gonzaga Tone
Publication date: 01-01-2012
Publisher: Springer-Verlag
Published in: Child's Nervous System / Issue 1/2012
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-011-1601-z

At a glance: The STEP trials

Springer Medicine

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

Abstract

Introduction

Method

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Introduction

Method

Results

Conclusion

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