التفاصيل البيبلوغرافية
| العنوان: |
Genetic Characterization, Current Model Systems and Prognostic Stratification in PAX Fusion-Negative vs. PAX Fusion-Positive Rhabdomyosarcoma. |
| المؤلفون: |
Dehner, Carina A.1 (AUTHOR) cdehner@wustl.edu, Armstrong, Amy E.2 (AUTHOR) armstrongae@wustl.edu, Yohe, Marielle3 (AUTHOR) marielle.yohe@nih.gov, Shern, Jack F.3 (AUTHOR) john.shern@nih.gov, Hirbe, Angela C.4,5 (AUTHOR) hirbea@wustl.edu |
| المصدر: |
Genes. Oct2021, Vol. 12 Issue 10, p1500. 1p. |
| مصطلحات موضوعية: |
*SARCOMA, *RHABDOMYOSARCOMA, *PROGNOSTIC models, *PROGNOSIS, *ADULTS, *TUMOR markers |
| مستخلص: |
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents and accounts for approximately 2% of soft tissue sarcomas in adults. It is subcategorized into distinct subtypes based on histological features and fusion status (PAX-FOXO1/VGLL2/NCOA2). Despite advances in our understanding of the pathobiological and molecular landscape of RMS, the prognosis of these tumors has not significantly improved in recent years. Developing a better understanding of genetic abnormalities and risk stratification beyond the fusion status are crucial to developing better therapeutic strategies. Herein, we aim to highlight the genetic pathways/abnormalities involved, specifically in fusion-negative RMS, assess the currently available model systems to study RMS pathogenesis, and discuss available prognostic factors as well as their importance for risk stratification to achieve optimal therapeutic management. [ABSTRACT FROM AUTHOR] |
| قاعدة البيانات: |
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