☆ 4.5 Review

Emerging Therapeutic Strategies for Diffuse Intrinsic Pontine Glioma: A Systematic Review

HEALTHCARE (2023)

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Hypofractionated Radiation Therapy For Diffuse Intrinsic Pontine Glioma: A Noninferiority Randomized Study Including 253 Children

Mohamed S. Zaghloul et al.

Summary: The study aimed to compare the effects of different fractionated radiotherapy regimens on pediatric diffuse intrinsic pontine glioma. The results showed that two hypofractionated radiotherapy regimens were noninferior to conventional fractionation. Age was an important factor affecting patient survival.

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GD2-CART cell therapy for H3K27M-mutated diffuse midline gliomas

Robbie G. Majzner et al.

Summary: Diffuse intrinsic pontine glioma (DIPG) and other H3K27M-mutated diffuse midline gliomas (DMGs) are lethal pediatric tumors of the central nervous system. GD2-directed chimeric antigen receptor (CAR) T cells show promising preclinical efficacy. Four patients with H3K27M-mutated DIPG or spinal cord DMG were treated with GD2-CAR T cells and showed clinical and radiographic improvement.

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Article Oncology

Phase I/II trial of vorinostat and radiation and maintenance vorinostat in children with diffuse intrinsic pontine glioma: A Children's Oncology Group report

Jack M. Su et al.

Summary: The phase I/II trial of vorinostat in children with newly diagnosed DIPG showed that the regimen of vorinostat given concurrently with radiation followed by vorinostat monotherapy was well tolerated but did not significantly improve outcomes.

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Article Oncology

Infusion-related side-effects during convection enhanced delivery for brainstem-diffuse midline glioma/diffuse intrinsic pontine glioma

Milo Hollingworth et al.

Summary: Side-effects during brainstem infusion are common and can be transient or persist longer than 24 hours. Neurological injury during infusion may be time-dependent and accumulative rather than volume-dependent.

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Oncolytic DNX-2401 Virus for Pediatric Diffuse Intrinsic Pontine Glioma

Jaime Gallego Perez-Larraya et al.

Summary: This study investigated the use of oncolytic virus DNX-2401 in pediatric patients with diffuse intrinsic pontine glioma (DIPG). The results showed that treatment with DNX-2401 led to changes in T-cell activity and reduction or stabilization of tumor size in some patients, but also caused adverse events.

NEW ENGLAND JOURNAL OF MEDICINE (2022)

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Article Oncology

Wee1 kinase inhibitor adavosertib with radiation in newly diagnosed diffuse intrinsic pontine glioma: A Children's Oncology Group phase I consortium study

Sabine Mueller et al.

Summary: The study evaluated the use of Adavosertib in combination with CRT in children with newly diagnosed DIPG, with the recommended phase 2 dose being 200 mg/m(2) per day. While the combination treatment was well tolerated, it did not improve overall survival compared to historical controls. These findings can provide insights for future trial designs in children with high-risk cancer.

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Article Oncology

Phase I trial of sargramostim/pelareorep therapy in pediatric patients with recurrent or refractory high-grade brain tumors

Matthew R. Schuelke et al.

Summary: This study reports a phase I trial evaluating the safety and immunologic responses to sargramostim/pelareorep in pediatric patients with recurrent or refractory high-grade brain tumors. The results showed that sargramostim/pelareorep had no significant treatment effect on the patients, but caused some adverse events. Although there are limitations to this study, it is of certain importance for the treatment research of pediatric brain tumors.

NEURO-ONCOLOGY ADVANCES (2022)

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Phase I study of ribociclib and everolimus in children with newly diagnosed DIPG and high-grade glioma: A CONNECT pediatric neuro-oncology consortium report

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CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014-2018

Quinn T. Ostrom et al.

Summary: The report from CBTRUS provides the most up-to-date population-based data on primary brain tumors in the United States, including incidence rates, mortality rates, and survival rates for both malignant and non-malignant tumors.

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Article Oncology

A phase I/II study of bevacizumab, irinotecan and erlotinib in children with progressive diffuse intrinsic pontine glioma

Fatma E. El-Khouly et al.

Summary: This study found that daily erlotinib is safe and well tolerated in doses up to 85 mg/m(2) when combined with biweekly bevacizumab and irinotecan in children with progressive DIPG. The median overall survival of the study patients was longer than known from literature.

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Article Clinical Neurology

Intraarterial delivery of bevacizumab and cetuximab utilizing blood-brain barrier disruption in children with high-grade glioma and diffuse intrinsic pontine glioma: results of a phase I trial

Heather J. McCrea et al.

Summary: Delivery of drugs intraarterially to brain tumors has been demonstrated in adults. In this study, the authors initiated a phase I trial of superselective intraarterial cerebral infusion (SIACI) of bevacizumab and cetuximab in pediatric patients with refractory high-grade glioma (diffuse intrinsic pontine glioma [DIPG] and glioblastoma) to determine the safety and efficacy in this population. SIACI was well tolerated in all 13 children, with some patients showing symptom improvement and decreased enhancement on MRI scans.

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Article Oncology

Phase 2 Study of Pomalidomide (CC-4047) Monotherapy for Children and Young Adults With Recurrent or Progressive Primary Brain Tumors

Jason Fangusaro et al.

Summary: The study investigating the safety and efficacy of pomalidomide in children and young adults with recurrent primary brain tumors did not meet the primary measure of success. Future studies are required to evaluate the effectiveness of POM in tumors with specific molecular signatures or in combination with other anticancer agents.

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Reirradiation practices for children with diffuse intrinsic pontine glioma

Chantel Cacciotti et al.

Summary: This study surveyed North American physicians on the practice of reirradiation for DIPG, showing that 88% of respondents consider reirradiation a treatment option, with most believing it should be done at least 6 months after initial RT. However, there was variation in total doses and fractionation, indicating the need for further clinical trials to determine optimal radiation strategies for children with progressive DIPG.

NEURO-ONCOLOGY PRACTICE (2021)

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Article Clinical Neurology

ABC Transporter Inhibition Plus Dexamethasone Enhances the Efficacy of Convection Enhanced Delivery in H3.3K27M Mutant Diffuse Intrinsic Pontine Glioma

Vadim Tsvankin et al.

NEUROSURGERY (2020)

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Hypofractionated radiotherapy with temozolomide in diffuse intrinsic pontine gliomas: a randomized controlled trial

Yousra Izzuddeen et al.

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A phase I/II study of veliparib (ABT-888) with radiation and temozolomide in newly diagnosed diffuse pontine glioma: a Pediatric Brain Tumor Consortium study

Patricia A. Baxter et al.

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A phase 2 study of valproic acid and radiation, followed by maintenance valproic acid and bevacizumab in children with newly diagnosed diffuse intrinsic pontine glioma or high-grade glioma

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Evan D. Bander et al.

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Combined treatment with CBP and BET inhibitors reverses inadvertent activation of detrimental super enhancer programs in DIPG cells

Maria Wiese et al.

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Nimotuzumab and radiotherapy for treatment of newly diagnosed diffuse intrinsic pontine glioma (DIPG): a phase III clinical study

G. Fleischhack et al.

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Phase I trial of convection-enhanced delivery of IL13-Pseudomonas toxin in children with diffuse intrinsic pontine glioma

John D. Heiss et al.

JOURNAL OF NEUROSURGERY-PEDIATRICS (2019)

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Diagnostics and treatment of diffuse intrinsic pontine glioma: where do we stand?

Fatma E. El-Khouly et al.

JOURNAL OF NEURO-ONCOLOGY (2019)

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Pediatric diffuse intrinsic pontine glioma: where do we stand?

Wafaa M. Rashed et al.

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A Phase 1/2 Trial of Reirradiation for Diffuse Intrinsic Pontine Glioma

Mark J. Amsbaugh et al.

INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS (2019)

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Role of Radiation Therapy in the Management of Diffuse Intrinsic Pontine Glioma: A Systematic Review

Matthew Gallitto et al.

ADVANCES IN RADIATION ONCOLOGY (2019)

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A pediatric brain tumor consortium phase II trial of capecitabine rapidly disintegrating tablets with concomitant radiation therapy in children with newly diagnosed diffuse intrinsic pontine gliomas

Lindsay B. Kilburn et al.

PEDIATRIC BLOOD & CANCER (2018)

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A phase 1/2 dose-finding, safety, and activity study of cabazitaxel in pediatric patients with refractory solid tumors including tumors of the central nervous system

Peter E. Manley et al.

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Reirradiation and PD-1 inhibition with nivolumab for the treatment of recurrent diffuse intrinsic pontine glioma: a single-institution experience

Cassie Kline et al.

JOURNAL OF NEURO-ONCOLOGY (2018)

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Survival benefit for patients with diffuse intrinsic pontine glioma (DIPG) undergoing re-irradiation at first progression: A matched-cohort analysis on behalf of the SIOP-E-HGG/DIPG working group

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