Genetic Characterization, Current Model Systems and Prognostic Stratification in PAX Fusion-Negative vs. PAX Fusion-Positive Rhabdomyosarcoma.
Genetic Characterization, Current Model Systems and Prognostic Stratification in PAX Fusion-Negative vs. PAX Fusion-Positive Rhabdomyosarcoma.
复制标题
PAX融合阴性与PAX融合阳性横纹肌肉瘤的遗传特征、当前模型系统和预后分层。
DOI：
10.3390/genes12101500
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发表时间：
2021-09-25
期刊：
影响因子：
3.5
通讯作者：
Hirbe AC
中科院分区：
文献类型：
--
作者：
Dehner CA;Armstrong AE;Yohe M;Shern JF;Hirbe AC
横纹肌肉瘤(RMS)是儿童和青少年最常见的软组织肉瘤，约占成人软组织肉瘤的2%。根据组织学特征和融合状态(PAX-FOXO1/VGLL2/NCOA2)将其分为不同的亚型。尽管我们对RMS的病理生物学和分子图景的了解取得了进展，但近年来这些肿瘤的预后并没有显著改善。更好地了解基因异常和融合状态以外的风险分层对于制定更好的治疗策略至关重要。在这里，我们的目的是强调涉及的遗传途径/异常，特别是在融合阴性的RMS，评估目前可用的模型系统来研究RMS的发病机制，并讨论可用的预后因素及其对风险分层的重要性，以实现最佳的治疗管理。
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.
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影响因子：
3.7
作者：
Bridge, JA;Liu, J;Barr, FG
通讯作者：
Barr, FG
影响因子：
7.3
作者：
Doyle, Brendan;Morton, Jennifer P.;Sansom, Owen J.
通讯作者：
Sansom, Owen J.
影响因子：
2.7
作者：
Flanigan, Jaclyn C.;Jilaveanu, Lucia B.;Kluger, Harriet M.
通讯作者：
Kluger, Harriet M.
影响因子：
--
作者：
Nitzki F;Tolosa EJ;Cuvelier N;Frommhold A;Salinas-Riester G;Johnsen SA;Fernandez-Zapico ME;Hahn H
通讯作者：
Hahn H
影响因子：
50.3
作者：
Drummond CJ;Hanna JA;Garcia MR;Devine DJ;Heyrana AJ;Finkelstein D;Rehg JE;Hatley ME
通讯作者：
Hatley ME