Article
Oncology
Olivia M. T. Davies, Anna L. Bruckner, Timothy McCalmont, Leo Mascarenhas, Vikash Oza, Mary L. Williams, Lara Wine-Lee, Jack F. Shern, Dawn H. Siegel
Summary: Variants in RAS genes are associated with the development of certain pediatric cancers, including rhabdomyosarcoma. Somatic RAS variants may increase the risk of rhabdomyosarcoma, and the presence of RAS-driven birthmarks could serve as a potential indicator of its development. Further research is needed to determine the incidence of rhabdomyosarcoma and establish monitoring guidelines for at-risk patients.
PEDIATRIC BLOOD & CANCER
(2022)
Article
Biochemistry & Molecular Biology
Christophe Glorieux, Xiaojun Xia, Yong-Qiao He, Yumin Hu, Kelly Cremer, Annie Robert, Junchen Liu, Fen Wang, Jianhua Ling, Paul J. Chiao, Peng Huang
Summary: K-ras mutations enhance PD-L1 expression through a redox-mediated mechanism, while inhibition of the FGFR1 pathway reduces PD-L1 expression and enhances T cell-mediated tumor suppression.
Article
Cell Biology
Kengo Nakahata, Brian W. Simons, Enrico Pozzo, Ryan Shuck, Lyazat Kurenbekova, Zachary Prudowsky, Kshiti Dholakia, Cristian Coarfa, Tajhal D. Patel, Lawrence A. Donehower, Jason T. Yustein
Summary: This study developed a genetically engineered mouse model (GEMM) for rhabdomyosarcoma (RMS) and found that the RMS cell lines derived from this model showed similar therapeutic responsiveness to treatment as human RMS cell lines. These findings provide an important tool for studying and evaluating new treatment modalities for RMS.
DISEASE MODELS & MECHANISMS
(2022)
Article
Multidisciplinary Sciences
Jenny J. Li, Alexander R. Kovach, Margaret DeMonia, Katherine K. Slemmons, Kristianne M. Oristian, Candy Chen, Corinne M. Linardic
Summary: Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma, with different prognoses and molecular characteristics between the embryonal and alveolar variants. A study found that oncogenic RAS expression is detrimental to cell growth and proliferation in aRMS, providing insight into potential therapeutic opportunities for different RMS subtypes.
SCIENTIFIC REPORTS
(2021)
Article
Chemistry, Multidisciplinary
Weiqi Cai, Tianli Luo, Xianghan Chen, Lanqun Mao, Ming Wang
Summary: This study reports the use of ROS-degradable lipid nanoparticles for delivering mRNA into tumor cells, resulting in enhanced gene expression. It was found that BAmP-TK-12 showed superior efficacy in tumor cells and could selectively deplete mutant RAS in tumor cells through the delivery of specific mRNA, leading to significantly improved antitumor effects.
ADVANCED FUNCTIONAL MATERIALS
(2022)
Article
Biochemistry & Molecular Biology
Julia Bauer, Nicole Cuvelier, Nada Ragab, Katja Simon-Keller, Frauke Nitzki, Natalie Geyer, Dominik S. Botermann, Dominik P. Elmer, Albert Rosenberger, Thomas A. Rando, Stefano Biressi, James A. Fagin, Dieter Saur, Christian Dullin, Hans-Ulrich Schildhaus, Walter Schulz-Schaeffer, Fritz Aberger, Anja Uhmann, Heidi Hahn
Summary: It has been found that in ERMS, the Hedgehog (HH)/GLI signaling pathway and RAS signaling pathway interact with each other, with RAS mutations able to increase proliferation and tumorigenicity by inhibiting the HH signaling pathway. In a mouse model, different RAS mutations have different effects on ERMS development.
Article
Biochemistry & Molecular Biology
Manuela Piazzi, Alberto Bavelloni, Vittoria Cenni, Sara Salucci, Anna Bartoletti Stella, Enrica Tomassini, Katia Scotlandi, William L. Blalock, Irene Faenza
Summary: In this study, BYL-719 inhibitor demonstrated anti-tumorgenic properties in treating rhabdomyosarcoma. When combined with CAL-101 inhibitor, BYL-719 showed synergistic effects in reducing cell viability and inducing apoptosis. These findings suggest that BYL-719, either alone or in combination with CAL-101, could be an efficient treatment for rhabdomyosarcoma with abnormal upregulation of the PI3K signaling pathway.
Article
Biochemistry & Molecular Biology
Ming-Ju Hsieh, Hsin-Yu Ho, Yu-Sheng Lo, Chia-Chieh Lin, Yi-Ching Chuang, Mosleh Mohammad Abomughaid, Ming-Chang Hsieh, Mu-Kuan Chen
Summary: The study investigates the anticancer activities of a natural compound called semilicoisoflavone B (SFB) in oral squamous cell carcinoma (OSCC). Results show that SFB reduces cell viability by targeting the cell cycle and inducing apoptosis. It also inhibits several signaling pathways and downregulates survivin expression. Overall, the study suggests that SFB has potential as a therapeutic agent for human OSCC.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Genetics & Heredity
Carina A. Dehner, Amy E. Armstrong, Marielle Yohe, Jack F. Shern, Angela C. Hirbe
Summary: Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents, but the prognosis has not significantly improved in recent years. Researchers are working to better understand the genetic abnormalities and risk stratification of the disease in order to develop more effective therapeutic strategies.
Article
Pathology
Joanna Cyrta, Arnaud Gauthier, Marie Karanian, Andre F. Vieira, Liesbeth Cardoen, Nina Jehanno, Megane Bouvet, Corinne Bouvier, Mina Komuta, Francois Le Loarer, Daniel Orbach, Angelique Rome, Veronique Minard-Colin, Benedicte Brichard, Claire Pluchart, Estelle Thebaud, Marleen Renard, Stephanie Pannier, Herve Brisse, Philippe Petit, Camille Benoist, Gudrun Schleiermacher, Birgit Geoerger, Anne Vincent-Salomon, Paul Freneaux, Gaelle Pierron
Summary: VGLL2-rearranged rhabdomyosarcomas are rare tumors with generally favorable outcomes, but some cases can experience local progression and distant metastases, highlighting the importance of complete surgical resection and consideration of RMS-type chemotherapy in unresectable cases.
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
(2021)
Article
Oncology
Gianni Bisogno, Joerg Fuchs, Roshni Dasgupta, Andrea Ferrari, Josephine H. Haduong, Timothy Rogers, David O. Walterhouse, Beatrice Coppadoro, Wei Xue, Christian Vokuhl, Douglas S. Hawkins, Guido Seitz, Johannes H. M. Merks, Monika Sparber-Sauer, Rajkumar Venkatramani
Summary: This study aimed to investigate the prognosis of completely resected embryonal RMS at sites other than paratesticular, uterine, or vaginal primary sites. The results showed that patients with nonalveolar RMS completely resected at diagnosis have excellent survival rates when arising from nonparatesticular, uterine, and vaginal sites, and can be successfully treated with low-intensity chemotherapy.
Article
Pathology
Xinyi Zhang, Shidan Wang, Erin R. Rudzinski, Saloni Agarwal, Ruichen Rong, Donald A. Barkauskas, Ovidiu Daescu, Lauren Furman Cline, Rajkumar Venkatramani, Yang Xie, Guanghua Xiao, Patrick Leavey
Summary: This study developed a computational algorithm for subtype classification and prognosis prediction of rhabdomyosarcoma (RMS) using pathology images. The algorithm achieved good performance in classification and prediction on the test dataset, providing assistance in pathology evaluation and risk stratification.
AMERICAN JOURNAL OF PATHOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Arathi Nair, Katharina F. Kubatzky, Bhaskar Saha
Summary: The Ras GTPase plays a central role in oncogenic circuitry, with its isoforms H-, K-, and N-Ras exhibiting functional differences and cell type-specific activities. The preferential localization of different Ras isoforms in various cellular compartments adds a new dimension to isoform-specific signaling and diverse functions. Therefore, considering isoform-specific properties in the design of therapeutic strategies is crucial for effective treatments in the future.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Editorial Material
Endocrinology & Metabolism
Philippe Icard, Marco Alifano, Emmanuel Donnadieu, Luca Simula
Summary: F-1,6-BP enhances aerobic glycolysis and sustains T cell activation while downregulating oxidative metabolism through a feedback loop between PFK1, PI3K/Akt, and PFK2/PFKFB3, which is also sustained by low citrate and mitochondrial ATP synthesis in cancer cells.
TRENDS IN ENDOCRINOLOGY AND METABOLISM
(2021)
Review
Oncology
Jennifer T. Castle, Brittany E. Levy, Derek B. Allison, David A. Rodeberg, Eric J. Rellinger
Summary: Rhabdomyosarcomas are soft tissue tumors commonly found in children, especially in the genitourinary tract. Treatment options, including chemotherapy and radiation, have improved outcomes and allowed for less invasive surgeries. This review provides an understanding of the management of these tumors, with a focus on those arising from the genitourinary tract.
