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 …

EANO-EURACAN-SNO Guidelines on circumscribed astrocytic gliomas, glioneuronal, and neuronal tumors

R Rudà, D Capper, AD Waldman, J Pallud… - Neuro …, 2022 - academic.oup.com
In the new WHO 2021 Classification of CNS Tumors the chapter “Circumscribed astrocytic
gliomas, glioneuronal and neuronal tumors” encompasses several different rare tumor …

The type II RAF inhibitor tovorafenib in relapsed/refractory pediatric low-grade glioma: the phase 2 FIREFLY-1 trial

LB Kilburn, DA Khuong-Quang, JR Hansford, D Landi… - Nature medicine, 2024 - nature.com
BRAF genomic alterations are the most common oncogenic drivers in pediatric low-grade
glioma (pLGG). Arm 1 (n= 77) of the ongoing phase 2 FIREFLY-1 (PNOC026) trial …

MAPK inhibitor sensitivity scores predict sensitivity driven by the immune infiltration in pediatric low-grade gliomas

R Sigaud, TK Albert, C Hess, T Hielscher… - Nature …, 2023 - nature.com
Pediatric low-grade gliomas (pLGG) show heterogeneous responses to MAPK inhibitors
(MAPKi) in clinical trials. Thus, more complex stratification biomarkers are needed to identify …

A phase II trial of selumetinib in children with recurrent optic pathway and hypothalamic low-grade glioma without NF1: a Pediatric Brain Tumor Consortium study

J Fangusaro, A Onar-Thomas, TY Poussaint… - Neuro …, 2021 - academic.oup.com
Background Pediatric low-grade gliomas (pLGGs) are the most common childhood brain
tumor. Progression-free survival (PFS) is much lower than overall survival, emphasizing the …

Response to trametinib treatment in progressive pediatric low-grade glioma patients

F Selt, CM van Tilburg, B Bison, P Sievers… - Journal of Neuro …, 2020 - Springer
Introduction A hallmark of pediatric low-grade glioma (pLGG) is aberrant signaling of the
mitogen activated protein kinase (MAPK) pathway. Hence, inhibition of MAPK signaling …

Efficacy and safety of larotrectinib in TRK fusion-positive primary central nervous system tumors

F Doz, CM Van Tilburg, B Geoerger… - Neuro …, 2022 - academic.oup.com
Background Larotrectinib is a first-in-class, highly selective tropomyosin receptor kinase
(TRK) inhibitor approved to treat adult and pediatric patients with TRK fusion-positive …

Automated tumor segmentation and brain tissue extraction from multiparametric MRI of pediatric brain tumors: A multi-institutional study

A Fathi Kazerooni, S Arif, R Madhogarhia… - Neuro-Oncology …, 2023 - academic.oup.com
Background Brain tumors are the most common solid tumors and the leading cause of
cancer-related death among all childhood cancers. Tumor segmentation is essential in …

A nationwide evaluation of bevacizumab-based treatments in pediatric low-grade glioma in the UK: safety, efficacy, visual morbidity, and outcomes

K Green, P Panagopoulou, F D'Arco, P O'Hare… - Neuro …, 2023 - academic.oup.com
Background Bevacizumab is increasingly used in children with pediatric low-grade glioma
(PLGG) despite limited evidence. A nationwide UK service evaluation was conducted to …

LOGGIC/FIREFLY-2: a phase 3, randomized trial of tovorafenib vs. chemotherapy in pediatric and young adult patients with newly diagnosed low-grade glioma …

CM van Tilburg, LB Kilburn, S Perreault, R Schmidt… - BMC cancer, 2024 - Springer
Background Pediatric low-grade glioma (pLGG) is essentially a single pathway disease, with
most tumors driven by genomic alterations affecting the mitogen-activated protein …