Dependencies & Vulnerabilities in SMARCB1-deficient Cancers

May 14-17, 2023
Saint-Paul-de-Vence, France


The ambition of the SMARCB1 Hope 2023 Symposium is to:

assess scientific progress — and failures — since the gene’s initial discovery

explore new approaches potentially conducive to effective therapies

highlight and find ways to overcome obstacles to the development of new therapies

foster international cooperation, research programs and the sharing of data and tissue banks

define future research priorities that will lead to private and public funding for projects



Olivier Delattre: Historical Background and Challenges Ahead

Chromatin Remodeling & Epigenetics – Towards Therapeutic Interventions

Charles Roberts: SMARCB1-deficient cancers: mechanisms and vulnerabilities
Matthieu Gérard: SMARCB1 and BRG1 generate subnucleosomes that expand transcription factor binding and function at enhancers
Irwin Davidson: BRG1 in oncogenesis, the example of melanoma
Cigall Kadoch: Structure and function of mSWI/SNF ATP-dependent chromatin remodeling complexes in SMARCB1-deficient cancers
Nada Jabado: PRC1, PRC2, BAF and COMPASS complexes: it takes more than 2 to tango in pediatric high grade brain tumors

From Cell-of-Origin to Transformed Cells

Steve Baylin: Roles for abnormal DNA methylation and chromatin remodeling in the origins of human cancer – from cell of origin to transformed cells
Sam Behjati: Phylogenetic approaches to finding the cell of origin of rhabdoid tumors
Jarno Drost: Patient-specific epigenetic reprogramming drives malignant rhabdoid tumorigenesis
Franck Bourdeaut: Cell-of-origin – what mouse models can teach us

Constellation of SMARCB1/A4-deficient Tumors – Overlaps and Specificities

William Foulkes: SMARCA4 – updates on associated phenotypes, plausible treatment targets and available resources
Sophie Postel-Vinay: Epithelioid sarcoma: exploring tumor heterogeneity
Thomas Grünewald: Functional and translational genomics of Epithelioid sarcoma
Charles Keller: Functional genomic analysis of epithelioid sarcoma reveals distinct proximal and distal subtype biology
Martin Hasselblatt: Rare SMARCB1-deficient tumors of the CNS: Misfits or potential clues to a better understanding of rhabdoid tumor biology?
Gabriel Malouf: Role of SMARCB1 alterations in renal medullary carcinomas
Josh Sommer: Accelerating rare cancer research — the Chordoma Foundation experience

Models, Stratification & Perspectives in Therapeutic Targetting

Annie Huang: ATRTs sub-grouping – sufficient for therapeutic stratification?
Marcel Kool: Single cell transcriptional profiling of ATRTs
Paul Huang: The proteomic landscape of SMARCB1-deficient cancers
Dan Williamson: Subgroup/location-independent prognostic signatures and their correlates in Malignant Rhabdoid Tumors
Mrinal Gounder: Clinical Trials in SMARCB1 altered cancers – Past and Future Directions
Alex Kentsis: Overcoming clinical resistance to EZH2 inhibition of rhabdoid tumors using rational epigenetic combination therapy
Susan Chi: EZH2 inhibition: the long journey of pediatric drug development
Michael Fruehwald: Risk stratification in rhabdoid tumors – the need for novel markers AND therapies
Ulrich Schüller: Comparative analysis of matched primary and relapsing atypical teratoid/rhabdoid tumors (AT/RT)

The Tumor Cell in its “Omic” & Microenvironment Contexts

Emmanuel Donnadieu: Imaging of failure and success of CAR T-cells in human tumors
Daniel de Carvalho: Viral mimicry response to EZH2 inhibition in SMARCB1-deficient cancers
Eliane Piaggio: Immunotherapy of RT: a translational approach
Josh Waterfall: Transcriptional dysregulation as a source of tumor specific antigens
David Sourdive: Smart cells : an industrial perspective in adoptive cancer immunotherapy

Breakout Brainstorming Sessions


Stephen Baylin
Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins University (United States)
Sam Behjati
Wellcome Sanger Institute (United Kingdom)
Franck Bourdeaut
Institut Curie (France)
Susan Chi
Dana-Farber Cancer Institute, Harvard Medical School (United States)
Irwin Davidson
Institut de Génétique et de Biologie Moléculaire et Cellulaire - IGBMC (France)
Daniel De Carvalho
Princess Margaret Cancer Centre (Canada)
Olivier Delattre
Institut Curie (France)
Emmanuel Donnadieu
Institut Cochin (France)
Jarno Drost
Princess Maxima Center for Pediatric Oncology (The Netherlands)
William Foulkes
McGill University (Canada)
Michael Frühwald
University Children's Hospital Augsburg (Germany)
Matthieu Gérard
Institute for Integrative Biology of the Cell - I2BC (France)
Mrinal Gounder
Memorial Sloan Kettering Cancer Center (United States)
Thomas Grünewald
German Cancer Research Center - DKFZ, Hopp Children's Cancer Center -KiTZ (Germany)
Martin Hasselblatt
Institute of Neuropathology, University Hospital Münster (Germany)
Paul Huang
The Institute of Cancer Research - ICR (United Kingdom)
Annie Huang 
The Hospital for Sick Children, University of Toronto (Canada)
Nada Jabado
McGill University (Canada)
Cigall Kadoch
Dana-Farber Cancer Institute, Howard Hughes Medical Institute, the Broad Institute of MIT and Harvard (United States)
Charles Keller
Children's Cancer Therapy Development Institute (United States)
Alex Kentsis
Memorial Sloan Kettering Cancer Center (United States)
Marcel Kool
Princess Maxima Center for Pediatric Oncology (The Netherlands), German Cancer Research Center - DKFZ, Hopp Children's Cancer Center -KiTZ (Germany)
Gabriel Malouf
Institut de Génétique et de Biologie Moléculaire et Cellulaire - IGBMC Institut de Cancérologie de Strasbourg - ICANS (France)
Eliane Piaggio
Institut Curie (France)
Sophie Postel-Vinay
Gustave Roussy (France)
Charles Roberts
St. Jude Children's Research Hospital (United States)
Ulrich Schüller
University Medical Center Hamburg-Eppendorf (Germany)
Josh Sommer
Chordoma Foundation (United States)
David Sourdive
Cellectis (France & United States)
Cassie & Jean-Baptiste Toulouse
Founders, SMARCB1 Hope (France & United States)
Joshua Waterfall
Institut Curie (France)
Dan Williamson
Wolfson Children's Cancer Centre, Newcastle University (United Kingdom)


Domaine du Mas de Pierre

2320 route des Serres,
06570 Saint-Paul de Vence