Immunogenicity of rhabdoid tumors
New therapeutic perspectives for rhabdoid tumors

Rhabdoid tumors are one of the tumors presenting the fewest mutations among all human cancers; their ability to induce an immune response was presumed low until this study.
An integrated analysis of the immune infiltrate using immunohistochemistry , RNAseq and methylation profiles of a large series of rhabdoid tumors showed that a large proportion of tumors are abundantly infiltrated by T cells and myeloid cells. Further, scRNAseq (single cell RNA seq) sequencing associated with the sequencing of the gene encoding the T cell receptor revealed clonal expansion of these T cells unexpectedly suggesting the presence of a specific immune response of the tumor.
Checkpoint blockade therapy in an experimental model induced the regression of established tumors and durable immune responses paving the way for new therapeutic approaches for children.
Le blocage des points de contrôle immunitaire effectué sur un modèle murin induit une régression de la tumeur et l’apparition d’une mémoire immune in vivo ouvrant la voie à de nouvelles approches thérapeutiques pour les enfants.
A collaboration between SIRIC teams
This work was led by the physician-research Amaury Leruste and is the fruit of collaboration between 3 SIRIC endorsed teams:
- Translational Research in Pediatric Oncology (RTOP)
- Translational Research in Immunotherapy (TransIm)
- Integrative functional genomics of cance (IFGC)
Find out more about the translational research teams endorsed by SIRIC
Yarmarkovich et al wrote a postive preview of the Leruste et al publication. Read the preview