ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolution

F Flavia W. de Faria N Nicole C. Riedel D Daniel Münter M Marta Interlandi C Carolin Göbel L Lea Altendorf M Mathis Richter V Viktoria Melcher C Christian Thomas R Rajanya Roy M Melanie Schoof I Ivan Bedzhov N Natalia Moreno M Monika Graf M Marc Hotfilder D Dörthe Holdhof W Wolfgang Hartmann A Ann-Katrin Bruns A Angela Brentrup F Friederike Liesche-Starnecker B Bruno Maerkl S Sarah Sandmann J Julian Varghese M Martin Dugas P Pedro H. Pinto S Sebastian T. Balbach I I-Na Lu C Claudia Rossig O Oliver Soehnlein A Aysegül Canak M Martin Ebinger M Martin Schuhmann J Jens Schittenhelm M Michael F. Frühwald U Ulrich Schüller T Thomas K. Albert C Carolin Walter J Jan M. Bruder K Kornelius Kerl

Abstract

Abstract Embryonal tumor with multilayered rosettes (ETMR) is a lethal embryonal brain tumor entity. To investigate the intratumoral heterogeneity and cellular communication in the tumor microenvironment (TME), we analyze in this work single-cell RNA sequencing of about 250,000 cells of primary human and murine ETMR, in vitro cultures, and a 3D forebrain organoid model of ETMR, supporting the main findings with immunohistochemistry and spatial transcriptomics of human tumors. We characterize three distinct malignant ETMR subpopulations - RG-like, NProg-like and NB-like - positioned within a putative neurodevelopmental hierarchy. We reveal PDGFRβ+ pericytes as key communication partners in the TME, contributing to stem cell signaling through extracellular matrix-mediated interactions with tumor cells. PDGF signaling is upregulated in chemoresistant RG-like cells in vivo and plays a role in recruiting pericytes to ETMR TME by finalizing a signaling cascade which promotes the differentiation of non-malignant radial glia cells, derived from our 3D model, into pericyte-like cells. Selective PDGFR-inhibition blocked the lineage differentiation into pericytes in vitro and reduced the tumor cell population in vivo. Targeting ETMR-pericyte interactions in the TME presents a promising therapeutic approach.

Article Details

Volume / Issue Vol. 16, Issue 1
Published June 25, 2025
ISSN 2041-1723
Publisher Nature Portfolio

Journal Info

Nature Communications

Nature Portfolio

ISSN: 2041-1723 Open Access Life Sciences

Authors (39)

F

Flavia W. de Faria

N

Nicole C. Riedel

D

Daniel Münter

M

Marta Interlandi

C

Carolin Göbel

L

Lea Altendorf

M

Mathis Richter

V

Viktoria Melcher

C

Christian Thomas

R

Rajanya Roy

M

Melanie Schoof

I

Ivan Bedzhov

N

Natalia Moreno

M

Monika Graf

M

Marc Hotfilder

D

Dörthe Holdhof

W

Wolfgang Hartmann

A

Ann-Katrin Bruns

A

Angela Brentrup

F

Friederike Liesche-Starnecker

B

Bruno Maerkl

S

Sarah Sandmann

J

Julian Varghese

M

Martin Dugas

P

Pedro H. Pinto

S

Sebastian T. Balbach

I

I-Na Lu

C

Claudia Rossig

O

Oliver Soehnlein

A

Aysegül Canak

M

Martin Ebinger

M

Martin Schuhmann

J

Jens Schittenhelm

M

Michael F. Frühwald

U

Ulrich Schüller

T

Thomas K. Albert

C

Carolin Walter

J

Jan M. Bruder

K

Kornelius Kerl