Neoplasia Special Edition: Reviews and Current Studies from CBTN & PNOC

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In recognition of Childhood Brain Tumor Awareness Month, CBTN is excited to highlight a special edition of Neoplasia, which features publications from the Children's Brain Tumor Network and Pacific Pediatric Neuro-Oncology Consortium.

In this special issue, CBTN and PNOC scientists and clinicians present the current state of translational and clinical research in pediatric neuro-oncology. The featured papers highlight the efforts of prominent international clinicians and researchers who are working towards establishing centralized data repositories and consortia. They show how an innovative approach to collaborative open science can be utilized to enhance the outcomes for children diagnosed with brain tumors.

Featured Articles

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The Children’s Brain Tumor Network (CBTN) - Accelerating Research in Pediatric Central Nervous System Tumors through Collaboration and Open Science

The Children's Brain Tumor Network (CBTN) is a group of 32 institutions working together to collect and distribute biospecimens and data for pediatric brain tumors through open-science research platforms. As of August 2022, CBTN has enrolled over 4700 subjects and collected over 65,000 biospecimen aliquots. They have developed over 80 preclinical models from collected tumors and have multi-omic data for over 1000 tumors and germline material.

View more HERE

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Pioneering Models of Pediatric Brain Tumors

Brain cancer is the leading cause of death in the pediatric population. To improve survival, preclinical models are crucial in understanding the genetic and functional mechanisms of tumors. Learn more about the different models used in pediatric brain tumor research and their potential impact on treatment in this article.

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Interdisciplinary care of children with diffuse midline glioma

Diffuse Midline Glioma (DMG) which includes Diffuse Intrinsic Pontine Glioma (DIPG) is an infiltrative tumor of the midline structures of the central nervous system that demonstrates an aggressive pattern of growth and has no known curative treatment. As these tumors progress, children experience ongoing neurological decline including inability to ambulate, swallow and communicate effectively.

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Pediatric low-grade glioma: Targeted therapeutics and clinical trials in the molecular era

Pediatric Low-Grade Gliomas (pLGGs) are a type of brain tumor found in children. This article outlines some of the recent advances in understanding the genetic changes that cause these tumors have led to new treatments that target specific genes. This has allowed doctors to offer targeted therapies to patients with pLGG, which were not possible before.

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Unsupervised machine learning using K-means identifies radiomic subgroups of pediatric low-grade gliomas that correlate with key molecular markers

This study aimed to investigate if an unsupervised machine learning approach based on radiomic features could reveal distinct imaging subtypes of pediatric low-grade gliomas (pLGGs). The researchers collected multi-parametric MR images from 157 patients with pLGGs and extracted quantitative radiomic features from the tumorous region. They then used K-means clustering to identify three distinct imaging-based subtypes.

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Primary Central Nervous System Germ Cell Tumors in Children and Young Adults: A Review of Controversies in Diagnostic and Treatment Approach

Primary central nervous system (CNS) germ cell tumors (GCT) are a rare heterogenous group of cancers, arising most commonly in the second decade of life. Through several clinical trials conducted around the world by various groups, the treatment approach for CNS GCT has advanced substantially with generally improved overall outcomes. In recent years, the goal of clinical trials has been focused on reduction of the radiotherapy burden and minimization of long-term toxicity.

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Retrospective dataset and survey analyses identify gaps in data collection for craniopharyngioma and priorities of patients and families affected by the disease

Craniopharyngioma is a rare type of brain tumor located in the suprasellar region of the brain, close to important structures like the pituitary gland. This study aimed to better understand patient outcomes associated with existing therapies and to inform new treatment strategies.

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Indigenous peoples and inclusion in clinical and genomic research: Understanding the history and navigating contemporary engagement

The survival rates for pediatric cancer patients have improved significantly, but there are still significant disparities among underrepresented racial and ethnic groups. To address this issue, the Pacific Pediatric Neuro-Oncology Consortium (PNOC) and Children's Brain Tumor Network (CBTN) created a Diversity, Equity, and Inclusion (DEI) working group in 2020.

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Current Studies and Future Directions for Medulloblastoma: A Review from the Pacific Pediatric Neuro-Oncology Consortium (PNOC) Disease Working Group

Medulloblastoma is a type of brain tumor that commonly affects children, and it includes different subtypes that have varying characteristics and outcomes. Even with current treatments, long-term survival rates are low, and survivors often face various health challenges.

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Past, present and future of Focused Ultrasound as an adjunct or complement to DIPG/DMG therapy: A consensus of the 2021 FUSF DIPG meeting

Diffuse Midline Glioma (DMG) is a type of brain tumor that mainly affects children and has limited treatment options and a poor prognosis. One of the major obstacles to treating DMG is the blood-brain barrier, which prevents most drugs from reaching the tumor. Focused Ultrasound (FUS) is a noninvasive medical technology that has been shown to temporarily and safely disrupt the blood-brain barrier.

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Locoregional CAR T cells for children with CNS tumors: Clinical procedure and catheter safety

This study discusses the use of chimeric antigen receptor (CAR) T cell therapy to treat pediatric central nervous system (CNS) tumors. These tumors are the most common solid malignancy in children, and CAR T cell therapy has shown success in treating childhood leukemia and preclinical efficacy against pediatric CNS tumors.

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Current state of pediatric neuro-oncology imaging, challenges and future directions

Imaging plays an important role in the diagnosis, treatment, and monitoring of brain tumors. With the development of new imaging technologies and data analysis methods, doctors can create large databases of brain images that can be used to improve patient care. In this review, researchers discusses various types of imaging techniques and their pros and cons, as well as efforts to standardize imaging practices.

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Recent progress and novel approaches to treating atypical teratoid rhabdoid tumor

Atypical teratoid rhabdoid tumors (AT/RT) are malignant central nervous system (CNS) tumors that occur mostly in young children and have historically carried a very poor prognosis.

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Myxoid glioneuronal tumor: Histopathologic, neuroradiologic, and molecular features in a single center series

Myxoid glioneuronal tumor (MGT) is a non-cancerous type of brain tumor that was recently added to the World Health Organization (WHO) classification of central nervous system (CNS) tumors. MGTs are usually found in specific areas of the brain, such as the septum pellucidum, foramen of Monro, or periventricular white matter of the lateral ventricle.

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Leptomeningeal Dissemination in Pediatric Brain Tumors

A condition called leptomeningeal disease (LMD) is not well understood in children with brain tumors. LMD is when the cancer cells spread to the tissues surrounding the brain and spinal cord. It can happen at the same time as the primary tumor, after the tumor comes back, or even without a tumor in the brain.

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The promise of metabolic imaging in diffuse midline glioma

Gliomas are a type of brain tumor that can be challenging to diagnose and treat, especially in children. Diffuse midline glioma (DMG) is a newly defined subtype that has a very poor prognosis. The diagnosis of DMG is based on clinical presentation and characteristic features on conventional magnetic resonance imaging (MRI).

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Developing H3K27M mutant selective radiosensitization strategies in diffuse intrinsic pontine glioma

This study examines a rare and deadly brain tumor called diffuse intrinsic pontine glioma (DIPG), which mainly affects children and teenagers. The current standard of care, which involves radiotherapy, has minimal benefit for DIPG patients, and efforts to combine chemotherapy with radiation therapy have been unsuccessful.

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Why haven’t we solved intracranial pediatric ependymoma? Current questions and barriers to treatment advances

Pediatric intracranial ependymoma, a type of brain tumor, has recently been divided into subgroups based on specific characteristics. This new information can make it more difficult to draw conclusions from past treatments, but it also presents an opportunity to better understand the disease and develop effective therapies.

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