Sarcoma treatment in the era of molecular medicine

TGP Grünewald, M Alonso, S Avnet… - EMBO molecular …, 2020 - embopress.org
Sarcomas are heterogeneous and clinically challenging soft tissue and bone cancers.
Although constituting only 1% of all human malignancies, sarcomas represent the second …

Rhabdomyosarcoma

SX Skapek, A Ferrari, AA Gupta, PJ Lupo… - Nature reviews disease …, 2019 - nature.com
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and
represents a high-grade neoplasm of skeletal myoblast-like cells. Decades of clinical and …

Vinorelbine and continuous low-dose cyclophosphamide as maintenance chemotherapy in patients with high-risk rhabdomyosarcoma (RMS 2005): a multicentre …

G Bisogno, GL De Salvo, C Bergeron… - The lancet …, 2019 - thelancet.com
Background For more than three decades, standard treatment for rhabdomyosarcoma in
Europe has included 6 months of chemotherapy. The European paediatric Soft tissue …

Single-cell analysis and functional characterization uncover the stem cell hierarchies and developmental origins of rhabdomyosarcoma

Y Wei, Q Qin, C Yan, MN Hayes, SP Garcia, H Xi, D Do… - Nature cancer, 2022 - nature.com
Rhabdomyosarcoma (RMS) is a common childhood cancer that shares features with
developing skeletal muscle. Yet, the conservation of cellular hierarchy with human muscle …

Single-cell profiling of alveolar rhabdomyosarcoma reveals RAS pathway inhibitors as cell-fate hijackers with therapeutic relevance

SG Danielli, E Porpiglia, AJ De Micheli, N Navarro… - Science …, 2023 - science.org
Rhabdomyosarcoma (RMS) is a group of pediatric cancers with features of developing
skeletal muscle. The cellular hierarchy and mechanisms leading to developmental arrest …

Primary intracranial spindle cell sarcoma with rhabdomyosarcoma-like features share a highly distinct methylation profile and DICER1 mutations

C Koelsche, M Mynarek, D Schrimpf, L Bertero… - Acta …, 2018 - Springer
Patients with DICER1 predisposition syndrome have an increased risk to develop
pleuropulmonary blastoma, cystic nephroma, embryonal rhabdomyosarcoma, and several …

Single-cell transcriptomics reveals immune suppression and cell states predictive of patient outcomes in rhabdomyosarcoma

J DeMartino, MT Meister, LL Visser, M Brok… - Nature …, 2023 - nature.com
Paediatric rhabdomyosarcoma (RMS) is a soft tissue malignancy of mesenchymal origin that
is thought to arise as a consequence of derailed myogenic differentiation. Despite intensive …

PAX3-FOXO1 dictates myogenic reprogramming and rhabdomyosarcoma identity in endothelial progenitors

MB Searcy, RK Larsen IV, BT Stevens, Y Zhang… - Nature …, 2023 - nature.com
Fusion-positive rhabdomyosarcoma (FP-RMS) driven by the expression of the PAX3-FOXO1
(P3F) fusion oncoprotein is an aggressive subtype of pediatric rhabdomyosarcoma. FP-RMS …

Mesenchymal tumor organoid models recapitulate rhabdomyosarcoma subtypes

MT Meister, MJA Groot Koerkamp… - EMBO Molecular …, 2022 - embopress.org
Rhabdomyosarcomas (RMS) are mesenchyme‐derived tumors and the most common
childhood soft tissue sarcomas. Treatment is intense, with a nevertheless poor prognosis for …

The myogenesis program drives clonal selection and drug resistance in rhabdomyosarcoma

AG Patel, X Chen, X Huang, MR Clay, NL Komarova… - Developmental cell, 2022 - cell.com
Rhabdomyosarcoma (RMS) is a pediatric cancer with features of skeletal muscle; patients
with unresectable or metastatic RMS fare poorly due to high rates of disease recurrence …