Pediatric low-grade glioma in the era of molecular diagnostics

S Ryall, U Tabori, C Hawkins - Acta neuropathologica communications, 2020 - Springer
Low grade gliomas are the most frequent brain tumors in children and encompass a
spectrum of histologic entities which are currently assigned World Health Organisation …

Neurofibromatosis type 1

DH Gutmann, RE Ferner, RH Listernick… - Nature Reviews …, 2017 - nature.com
Neurofibromatosis type 1 is a complex autosomal dominant disorder caused by germline
mutations in the NF1 tumour suppressor gene. Nearly all individuals with neurofibromatosis …

Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre …

J Fangusaro, A Onar-Thomas, TY Poussaint… - The lancet …, 2019 - thelancet.com
Background Paediatric low-grade glioma is the most common CNS tumour of childhood.
Although overall survival is good, disease often recurs. No single universally accepted …

Health supervision for children with neurofibromatosis type 1

DT Miller, D Freedenberg, E Schorry, NJ Ullrich… - …, 2019 - publications.aap.org
Neuroibromatosis type 1 (NF1) is a multisystem disorder that primarily involves the skin and
peripheral nervous system. Its population prevalence is approximately 1 in 3000. The …

Malignant peripheral nerve sheath tumor: models, biology, and translation

BN Somatilaka, A Sadek, RM McKay, LQ Le - Oncogene, 2022 - nature.com
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, invasive cancer that
comprise around 10% of all soft tissue sarcomas and develop in about 8–13% of patients …

ERN GENTURIS tumour surveillance guidelines for individuals with neurofibromatosis type 1

C Carton, DG Evans, I Blanco, RE Friedrich… - …, 2023 - thelancet.com
Summary Background Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder,
predisposing development of benign and malignant tumours. Given the oncogenic potential …

Response assessment in paediatric low-grade glioma: recommendations from the Response Assessment in Pediatric Neuro-Oncology (RAPNO) working group

J Fangusaro, O Witt, PH Driever, AK Bag… - The lancet …, 2020 - thelancet.com
Paediatric low-grade gliomas (also known as pLGG) are the most common type of CNS
tumours in children. In general, paediatric low-grade gliomas show clinical and biological …

MEK inhibitors for neurofibromatosis type 1 manifestations: Clinical evidence and consensus

PMK De Blank, AM Gross, S Akshintala… - Neuro …, 2022 - academic.oup.com
The wide variety of clinical manifestations of the genetic syndrome neurofibromatosis type 1
(NF1) are driven by overactivation of the RAS pathway. Mitogen-activated protein kinase …

Optic pathway gliomas in neurofibromatosis‐1: controversies and recommendations

R Listernick, RE Ferner, GT Liu… - Annals of Neurology …, 2007 - Wiley Online Library
Optic pathway glioma (OPG), seen in 15% to 20% of individuals with neurofibromatosis type
1 (NF1), account for significant morbidity in young children with NF1. Overwhelmingly a …

[HTML][HTML] Neurofibromatosis 1

JM Friedman - 2022 - europepmc.org
Neurofibromatosis 1 (NF1) is a multisystem disorder characterized by multiple café au lait
macules, intertriginous freckling, multiple cutaneous neurofibromas, and learning disability …