AI Article Synopsis

  • * Traditional treatments for NF1-LGG have included chemotherapy and surgery, but some kids don’t respond well and may continue to struggle or see their condition worsen.
  • * New research is focusing on targeted therapies that aim to provide better treatment options, with ongoing studies looking into these innovative approaches and how to effectively manage and monitor NF1-LGG in children.

Article Abstract

Neurofibromatosis type 1 (NF1) is an autosomal dominant cancer predisposition syndrome characterized by the development of both central and peripheral nervous system tumors. Low-grade glioma (LGG) is the most prevalent central nervous system tumor occurring in children with NF1, arising most frequently within the optic pathway, followed by the brainstem. Historically, treatment of NF1-LGG has been limited to conventional cytotoxic chemotherapy and surgery. Despite treatment with chemotherapy, a subset of children with NF1-LGG fail initial therapy, have a continued decline in function, or recur. The recent development of several preclinical models has allowed for the identification of novel, molecularly targeted therapies. At present, exploration of these novel precision-based therapies is ongoing in the preclinical setting and through larger, collaborative clinical trials. Herein, we review the approach to surveillance and management of NF1-LGG in children and discuss upcoming novel therapies and treatment protocols.

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00381-024-06430-8DOI Listing

Publication Analysis

Top Keywords

low-grade glioma
8
neurofibromatosis type
8
nervous system
8
children
4
glioma children
4
children neurofibromatosis
4
type surveillance
4
treatment
4
surveillance treatment
4
treatment indications
4

Similar Publications

The role of MEK inhibition in pediatric low-grade gliomas.

Front Oncol

December 2024

Department of Pediatric Hematology-Oncology and Blood and Marrow Transplant, Cleveland Clinic, Cleveland, OH, United States.

Pediatric low-grade gliomas (pLGGs) are the most common brain tumors in children. Many patients with unresectable tumors experience recurrence or long-term sequelae from standard chemotherapeutics. This mini-review explores the emerging role of MEK inhibitors in the management of pLGGs, highlighting their potential to transform current treatment paradigms.

View Article and Find Full Text PDF

PET/CT targeting prostate-specific membrane antigen (PSMA) is commonly used in patients with prostate cancer. PSMA has been found in other solid tumours, including primary brain tumours. The aim of this study was to evaluate the usefulness of [Ga]Ga-PSMA-11 PET/CT for preoperative diagnosis and 2-year prognosis.

View Article and Find Full Text PDF

Unlabelled: QUESTIONS AND RECOMMENDATIONS FROM THE PRIOR VERSION OF THESE GUIDELINES WITHOUT CHANGE: TARGET POPULATION: Adult patients (age ≥ 18 years) who have suspected low-grade diffuse glioma.

Question: What are the optimal neuropathological techniques to diagnose low-grade diffuse glioma in the adult?

Recommendation: Level I Histopathological analysis of a representative surgical sample of the lesion should be used to provide the diagnosis of low-grade diffuse glioma. Level III Both frozen section and cytopathologic/smear evaluation should be used to aid the intra-operative assessment of low-grade diffuse glioma diagnosis.

View Article and Find Full Text PDF
Article Synopsis
  • Pediatric low-grade gliomas (pLGGs) show varying treatment responses and poor outcomes when complete tumor removal isn't possible, making early treatment prediction important.
  • A radiogenomic analysis combining MRI and RNA sequencing reveals three immune clusters in pLGGs, with one cluster having higher immune activity but worse prognosis, suggesting they might benefit from immunotherapy.
  • A developed radiomic signature accurately predicts these immune profiles and progression-free survival, identifying high-risk patients for potential targeted therapies.
View Article and Find Full Text PDF

Purpose: This study aims to summarize the characteristics of children under three years old (≤3 years) with central nervous system (CNS) tumors and to investigate the factors that influence their overall survival (OS) time.

Methods: We treated 171 pediatric patients (≤3 years) with CNS tumors at Yuquan Hospital of Tsinghua University from January 2016 to June 2023. Of these, 162 cases were successfully followed up.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!