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• W3002151685 endingPage "642" @default.
• W3002151685 startingPage "625" @default.
• W3002151685 abstract "Abstract While sarcomas account for approximately 1% of malignant tumors of adults, they are particularly more common in children and adolescents affected by cancer. In contrast to malignancies that occur in later stages of life, childhood tumors, including sarcoma, are characterized by a striking paucity of somatic mutations. However, entity-defining fusion oncogenes acting as the main oncogenic driver mutations are frequently found in pediatric bone and soft-tissue sarcomas such as Ewing sarcoma ( EWSR1-FLI1 ), alveolar rhabdomyosarcoma ( PAX3/7-FOXO1 ), and synovial sarcoma ( SS18-SSX1/2/4 ). Since strong oncogene-dependency has been demonstrated in these entities, direct pharmacological targeting of these fusion oncogenes has been excessively attempted, thus far, with limited success. Despite apparent challenges, our increasing understanding of the neomorphic features of these fusion oncogenes in conjunction with rapid technological advances will likely enable the development of new strategies to therapeutically exploit these neomorphic features and to ultimately turn the “undruggable” into first-line target structures. In this review, we provide a broad overview of the current literature on targeting neomorphic features of fusion oncogenes found in Ewing sarcoma, alveolar rhabdomyosarcoma, and synovial sarcoma, and give a perspective for future developments." @default.
• W3002151685 created "2020-01-30" @default.
• W3002151685 creator A5002454770 @default.
• W3002151685 creator A5005128186 @default.
• W3002151685 creator A5010237107 @default.
• W3002151685 creator A5011137037 @default.
• W3002151685 creator A5034606670 @default.
• W3002151685 creator A5068530878 @default.
• W3002151685 date "2019-12-01" @default.
• W3002151685 modified "2023-09-26" @default.
• W3002151685 title "Targeting the undruggable: exploiting neomorphic features of fusion oncoproteins in childhood sarcomas for innovative therapies" @default.
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