Dipg-55. Patterns of Cerebrospinal Fluid Diversion and Survival in Children With Diffuse Intrinsic Pontine Glioma: A Report From the International Dipg Registry

Cooney, Tabitha; DeWire-Schottmiller, Mariko; Lane, Adam; Saab, Raya; Bandopadhayay, Pratiti; Dorris, Kathleen; Packer, Roger J.; Kilburn, Lindsey; Minturn, Jane E.; Dodgshun, Andrew; Parkin, Sara; Goldman, Stewart; Sandler, Eric; Greiner, Robert; Gottardo, Nicholas; Dholaria, Hetal; Coven, Scott; Hassall, Tim; Hansford, Jordan R.; Samson, Yvan; Leary, Sarah; Bartels, Ute; Bouffet, Éric; Tinkle, Christopher L.; Monje, Michelle; Fisher, Paul; Ziegler, David S.; Chintagumpala, Murali; Wagner, Lars M.; Koschmann, Carl; Benito, Elisa Carrasquedo; Leach, James; Jones, Blaise V.; Chaney, Brooklyn; Black, Katie; Asher, Anthony L.; Bond, Hailey; Fouladi, Maryam; Warren, Katherine E.

doi:10.1093/neuonc/noaa222.100

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“…Due to the tumour location in the pons, which controls essential physiological functions, including breathing and heart rate, and the potential for significant and permanent morbidities affecting quality of life, or death, there is no role for surgical excision in patients with DIPG 47 . We do not recommend surgical intervention apart from biopsy or, in some cases, insertion of a ventriculoperitoneal shunt or reservoir 48 …”

Section: Recommendationsmentioning

confidence: 98%

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Management of patients with diffuse intrinsic pontine glioma in Australia and New Zealand: Australian and New Zealand Children's Haematology/Oncology Group position statement

Valvi,

Manoharan,

Mateos

et al. 2024

Medical Journal of Australia

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IntroductionThe main mission of the Australian and New Zealand Children's Haematology and Oncology Group (ANZCHOG) is to develop and facilitate local access to the world's leading evidence‐based clinical trials for all paediatric cancers, including brain tumours, as soon as practically possible. Diffuse intrinsic pontine gliomas (DIPGs) — a subset of a larger group of tumours now termed diffuse midline glioma, H3K27‐altered (DMG) — are paediatric brain cancers with less than 10% survival at two years. In the absence of any proven curative therapies, significant recent advancements have been made in pre‐clinical and clinical research, leading many to seek integration of novel therapies early into standard practice. Despite these innovative therapeutic approaches, DIPG remains an incurable disease for which novel surgical, imaging, diagnostic, radiation and systemic therapy approaches are needed.Main recommendations All patients with DIPG should be discussed in multidisciplinary neuro‐oncology meetings (including pathologists, neuroradiologists, radiation oncologists, neurosurgeons, medical oncologists) at diagnosis and at relapse or progression. Radiation therapy to the involved field remains the local and international standard of care treatment. Proton therapy does not yield a superior survival outcome compared with photon therapy and patients should undergo radiation therapy with the available modality (photon or proton) at their treatment centre. Patients may receive concurrent chemotherapy or radiation‐sensitising agents as part of a clinical trial. Biopsy should be offered to facilitate consideration of experimental therapies and eligibility for clinical trial participation. After radiation therapy, each patient should be managed individually with either observation or considered for enrolment on a clinical trial, if eligible, after full discussion with the family. Re‐irradiation can be considered for progressive disease. Changes in management as a result of the guidelineEvery child diagnosed with DIPG should be offered enrolment on a clinical trial where available. Access to investigational drugs without biological rationale outside the clinical trial setting is not supported. In case of potentially actionable target identification with molecular profiling and absence of a suitable clinical trial, rational targeted therapies can be considered through compassionate access programs.

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Section: Recommendationsmentioning

confidence: 98%

“…47 We do not recommend surgical intervention apart from biopsy or, in some cases, insertion of a ventriculoperitoneal shunt or reservoir. 48 Use of dexamethasone and bevacizumab. Dexamethasone is commonly used for symptomatic management by reducing oedema surrounding the tumour.…”

Section: Typical Imaging Features Of Diffuse Intrinsic Pontine Glioma...mentioning

confidence: 99%

Management of patients with diffuse intrinsic pontine glioma in Australia and New Zealand: Australian and New Zealand Children's Haematology/Oncology Group position statement

Valvi,

Manoharan,

Mateos

et al. 2024

Medical Journal of Australia

View full text Add to dashboard Cite

show abstract

Dipg-55. Patterns of Cerebrospinal Fluid Diversion and Survival in Children With Diffuse Intrinsic Pontine Glioma: A Report From the International Dipg Registry

Cited by 1 publication

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Management of patients with diffuse intrinsic pontine glioma in Australia and New Zealand: Australian and New Zealand Children's Haematology/Oncology Group position statement

Management of patients with diffuse intrinsic pontine glioma in Australia and New Zealand: Australian and New Zealand Children's Haematology/Oncology Group position statement

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