Real-time drug testing of paediatric diffuse midline glioma to support clinical decision making: The Zurich DIPG/DMG centre experience.

Brainstem biopsy Diffuse intrinsic pontine glioma Diffuse midline glioma Neurosurgery Paediatric brain cancer Patient derived cell lines Preclinical Targeted therapy Translational

Journal

European journal of cancer (Oxford, England : 1990)
ISSN: 1879-0852
Titre abrégé: Eur J Cancer
Pays: England
ID NLM: 9005373

Informations de publication

Date de publication:
01 2023
Historique:
received: 30 07 2022
revised: 28 09 2022
accepted: 17 10 2022
pubmed: 2 12 2022
medline: 21 12 2022
entrez: 1 12 2022
Statut: ppublish

Résumé

Children diagnosed with diffuse midline gliomas (DMG) have an extremely poor overall survival: 9-12 months from diagnosis with currently no curative treatment options. Given DMG molecular heterogeneity, surgical biopsies are needed for molecular profiling and as part of enrolment into molecular-based and precision medicine type clinical interventions. In this study, we describe the results of real time profiling and drug testing at the diffuse intrinsic pontine glioma/DMG Research Centre at University Children's Hospital Zurich. Biopsies were taken using a frame based stereotactic robot system (NeuroMate®, Renishaw) at University Children's Hospital Zurich. Tissue samples were evaluated to confirm diagnosis by H3K27M and H3K27 trimethylation loss. Genomic analyses were done using a variety of platforms (INFORM, Oncomine, UCSF500 gene panel). Cell lines were developed by mechanical tissue dissociation and verified by either sequencing or immunofluorescence staining confirming H3K27M mutation and used afterwards for drug testing. Twenty-five robot-assisted primary biopsies were successfully performed. Median hospital stay was 2 days (range 1-4 days). Nine low-passage patient-derived cells were developed, whereas 8 cell lines were used to inform response to clinically relevant drugs. Genome and RNA expression were used to further guide treatment strategies with targeted agents such as dual PI3K/mTOR inhibitor paxalisib. We established a systematic workflow for safe, robot-assisted brainstem biopsies and in-house tissue processing, followed by real-time drug testing. This provides valuable insights into tumour prognostic and individual treatment strategies targeting relevant vulnerabilities in these tumours in a clinically meaningful time frame.

Sections du résumé

BACKGROUND
Children diagnosed with diffuse midline gliomas (DMG) have an extremely poor overall survival: 9-12 months from diagnosis with currently no curative treatment options. Given DMG molecular heterogeneity, surgical biopsies are needed for molecular profiling and as part of enrolment into molecular-based and precision medicine type clinical interventions. In this study, we describe the results of real time profiling and drug testing at the diffuse intrinsic pontine glioma/DMG Research Centre at University Children's Hospital Zurich.
METHOD
Biopsies were taken using a frame based stereotactic robot system (NeuroMate®, Renishaw) at University Children's Hospital Zurich. Tissue samples were evaluated to confirm diagnosis by H3K27M and H3K27 trimethylation loss. Genomic analyses were done using a variety of platforms (INFORM, Oncomine, UCSF500 gene panel). Cell lines were developed by mechanical tissue dissociation and verified by either sequencing or immunofluorescence staining confirming H3K27M mutation and used afterwards for drug testing.
RESULTS
Twenty-five robot-assisted primary biopsies were successfully performed. Median hospital stay was 2 days (range 1-4 days). Nine low-passage patient-derived cells were developed, whereas 8 cell lines were used to inform response to clinically relevant drugs. Genome and RNA expression were used to further guide treatment strategies with targeted agents such as dual PI3K/mTOR inhibitor paxalisib.
CONCLUSION
We established a systematic workflow for safe, robot-assisted brainstem biopsies and in-house tissue processing, followed by real-time drug testing. This provides valuable insights into tumour prognostic and individual treatment strategies targeting relevant vulnerabilities in these tumours in a clinically meaningful time frame.

Identifiants

pubmed: 36455411
pii: S0959-8049(22)01309-0
doi: 10.1016/j.ejca.2022.10.014
pii:
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

171-179

Informations de copyright

Copyright © 2022. Published by Elsevier Ltd.

Déclaration de conflit d'intérêts

Conflict of interest statement We declare that we have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Auteurs

Timothy Mueller (T)

Division of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Sandra Laternser (S)

Division of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Ana S Guerreiro Stücklin (AS)

Division of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Nicolas U Gerber (NU)

Division of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Sulayman Mourabit (S)

Division of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Marion Rizo (M)

Division of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Elisabeth J Rushing (EJ)

Division of Neuropathology, University Hospital Zurich, Zurich, Switzerland.

Raimund Kottke (R)

Department of Diagnostic Imaging, University Children's Hospital Zurich, Zurich, Switzerland.

Michael Grotzer (M)

Division of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Niklaus Krayenbühl (N)

Department of Neurosurgery, University Children's Hospital Zurich, Zurich, Switzerland.

Javad Nazarian (J)

Division of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland; Children's National Health System, Center for Genetic Medicine Research, Washington, DC, 20010, USA.

Sabine Mueller (S)

Division of Oncology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland; Department Neurology, Neurosurgery, and Pediatrics, University of California San Francisco, San Francisco, CA, 94158, USA. Electronic address: Sabine.Mueller@ucsf.edu.

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Classifications MeSH