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Review: Ewing Sarcoma Predisposition

  • Review
  • Published:
Pathology & Oncology Research

Abstract

Ewing sarcoma is a rare tumor developed in bone and soft tissues of children and teenagers. This entity is biologically led by a chromosomal translocation, typically including EWS and FLI1 genes. Little is known about Ewing sarcoma predisposition, although the role of environmental factors, ethnicity and certain polymorphisms on Ewing sarcoma susceptibility has been studied during the last few years. Its prevalence among cancer predisposition syndromes has also been thoroughly examined. This review summarizes the available evidence on predisposing factors involved in Ewing sarcoma susceptibility. On the basis of these data, an integrated approach of the most influential factors on Ewing sarcoma predisposition is proposed.

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Abbreviations

ES:

Ewing sarcoma

CNV:

Copy number variations

CPS:

Cancer predisposing syndromes

MSC:

Mesenchymal Stem Cell

AACR:

American Association of Cancer Research

LFS:

Li-Fraumeni Syndrome

WGS:

whole genome sequencing

WES:

Whole exome sequencing

NGS:

Next generation sequencing

RB1 :

RB transcriptional corepressor 1

BLM :

Bloom Syndrome RecQ Like Helicase gene; Bloom syndrome gene

RET :

RET Proto-Oncogene

GENESIS:

Genetics of Ewing Sarcoma International study

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Acknowledgments

Arash Javadinejad (Health Institute La Fe, Valencia, Spain): English review and editing. Loreto Sales Triguero: graphic design of Fig. 1.

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Authors and Affiliations

  1. Clinical and Translational Oncology Research Group, La Fe Hospital, Av. Fernando Abril Martorell 106 Postal Code, 46026, Valencia, Spain

    Pablo Gargallo, Yania Yáñez & Vanessa Segura

  2. Pediatric Oncology and Hematology Unit, La Fe Hospital, Valencia, Spain

    Antonio Juan, Julia Balaguer, Bárbara Torres, Victoria Castel & Adela Cañete

  3. Genetics Unit, La Fe Hospital, Valencia, Spain

    Silves Oltra

  4. Genetics Department, Valencia University, Valencia, Spain

    Silves Oltra

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  1. Pablo Gargallo
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Gargallo, P., Yáñez, Y., Juan, A. et al. Review: Ewing Sarcoma Predisposition. Pathol. Oncol. Res. 26, 2057–2066 (2020). https://doi.org/10.1007/s12253-019-00765-3

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