Biological and therapeutic insights from animal modeling of fusion-driven pediatric soft tissue sarcomas

JP Kucinski, D Calderon… - Disease Models & …, 2024 - journals.biologists.com
Survival for children with cancer has primarily improved over the past decades due to
refinements in surgery, radiation and chemotherapy. Although these general therapies are …

Single cell transcriptomic profiling identifies tumor-acquired and therapy-resistant cell states in pediatric rhabdomyosarcoma

SG Danielli, Y Wei, MA Dyer, E Stewart… - Nature …, 2024 - nature.com
Rhabdomyosarcoma (RMS) is a pediatric tumor that resembles undifferentiated muscle
cells; yet the extent to which cell state heterogeneity is shared with human development has …

Dependence of PAX3-FOXO1 chromatin occupancy on ETS1 at important disease-promoting genes exposes new targetable vulnerability in Fusion-Positive …

J Hsieh, EP Danis, CR Owens, JK Parrish, NL Nowling… - Oncogene, 2024 - nature.com
Rhabdomyosarcoma (RMS), a malignancy of impaired myogenic differentiation, is the most
common soft tissue pediatric cancer. PAX3-FOXO1 oncofusions drive the majority of the …

Pediatric Tumors as Disorders of Development: The Case for In Vitro Modeling Based on Human Stem Cells

CD Clairmont, JJ Gell, CC Lau - Cancer Control, 2024 - journals.sagepub.com
Despite improvements in patient outcomes, pediatric cancer remains a leading cause of non-
accidental death in children. Recent genetic analysis of patients with pediatric cancers …

Epigenetic determinants of fusion-driven sarcomas: paradigms and challenges

BZ Stanton, S Pomella - Frontiers in Cell and Developmental Biology, 2024 - frontiersin.org
We describe exciting recent advances in fusion-driven sarcoma etiology, from an
epigenetics perspective. By exploring the current state of the field, we identify and describe …

[HTML][HTML] Novel PAX3:: MAML3 fusion identified in alveolar rhabdomyosarcoma, using DNA methylation profiling to expand the genetic spectrum of “fusion-positive” …

JK Dermawan, F Malik, JM Gross, E Baraban… - Modern Pathology, 2024 - Elsevier
Alveolar rhabdomyosarcoma (ARMS) with FOXO1 gene rearrangements is an aggressive
pediatric rhabdomyosarcoma subtype that is prognostically distinct from embryonal …

[HTML][HTML] Spatial Transcriptomics Analysis: Maternal Obesity Impairs Myogenic Cell Migration and Differentiation during Embryonic Limb Development

Y Gao, MN Hossain, L Zhao, JM Deavila… - International Journal of …, 2024 - mdpi.com
Limb muscle is responsible for physical activities and myogenic cell migration during
embryogenesis is indispensable for limb muscle formation. Maternal obesity (MO) impairs …

A Pax3 lineage gives rise to transient haematopoietic progenitors

G Canu, R Correra, G Diez-Pinel… - …, 2024 - journals.biologists.com
During embryonic development, muscle tissues, skin, and a subset of vascular endothelial
cells arise from Pax3-expressing embryonic progenitors defined as paraxial mesoderm. By …

Developmental Heterogeneity of Rhabdomyosarcoma

BT Stevens, ME Hatley - Cold Spring …, 2025 - perspectivesinmedicine.cshlp.org
Rhabdomyosarcoma (RMS) is a pediatric embryonal solid tumor and the most common
pediatric soft tissue sarcoma. The histology and transcriptome of RMS resemble skeletal …

Rhabdomyosarcoma fusion oncoprotein initially pioneers a neural signature in vivo

J Kucinski, A Tallan, C Taslim, M Wang, MV Cannon… - bioRxiv, 2024 - biorxiv.org
Fusion-positive rhabdomyosarcoma is an aggressive pediatric cancer molecularly
characterized by arrested myogenesis. The defining genetic driver, PAX3:: FOXO1, functions …