Precision medicine in Ewing sarcoma: a translational point of view

Ewing sarcoma is a rare tumor that arises in bones of children and teenagers but, in 15% of the patients it is presented as a primary soft tissue tumor. Balanced reciprocal chimeric translocation t (11;22)(q24;q12), which encodes an oncogenic protein fusion (EWSR1/FLI1), is the most generalized and...

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Bibliographic Details
Published in:Clinical & translational oncology 2020-09, Vol.22 (9), p.1440-1454
Main Authors: Gargallo, P., Juan, A., Yáñez, Y., Dolz, S., Segura, V., Castel, V., Cañete, A.
Format: Article
Language:English
Subjects:
Medicine
Medicine & Public Health
Oncology
Review Article
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Summary:Ewing sarcoma is a rare tumor that arises in bones of children and teenagers but, in 15% of the patients it is presented as a primary soft tissue tumor. Balanced reciprocal chimeric translocation t (11;22)(q24;q12), which encodes an oncogenic protein fusion (EWSR1/FLI1), is the most generalized and characteristic molecular event. Using conventional treatments, (chemotherapy, surgery and radiotherapy) long-term overall survival rate is 30% for patients with disseminated disease and 65–75% for patients with localized tumors. Urgent new effective drug development is a challenge. This review summarizes the preclinical and clinical investigational knowledge about prognostic and targetable biomarkers in Ewing sarcoma, finally suggesting a workflow for precision medicine committees.
ISSN:1699-048X
1699-3055
DOI:10.1007/s12094-020-02298-7