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Ewing Sarcoma: Current Management and Future Approaches Through Collaboration.

Gaspar, Nathalie; Hawkins, Douglas S; Dirksen, Uta; Lewis, Ian J; Ferrari, Stefano; Le Deley, Marie-Cecile; Kovar, Heinrich; Grimer, Robert; Whelan, Jeremy and Claude, Line, et al. (2015) In Journal of Clinical Oncology 33(27). p.140-3036
Abstract
Ewing sarcoma (ES) is an aggressive sarcoma of bone and soft tissue occurring at any age with a peak incidence in adolescents and young adults. The treatment of ES relies on a multidisciplinary approach, coupling risk-adapted intensive neoadjuvant and adjuvant chemotherapies with surgery and/or radiotherapy for control of the primary site and possible metastatic disease. The optimization of ES multimodality therapeutic strategies has resulted from the efforts of several national and international groups in Europe and North America and from cooperation between pediatric and medical oncologists. Successive first-line trials addressed the efficacy of various cyclic combinations of drugs incorporating doxorubicin, vincristine,... (More)
Ewing sarcoma (ES) is an aggressive sarcoma of bone and soft tissue occurring at any age with a peak incidence in adolescents and young adults. The treatment of ES relies on a multidisciplinary approach, coupling risk-adapted intensive neoadjuvant and adjuvant chemotherapies with surgery and/or radiotherapy for control of the primary site and possible metastatic disease. The optimization of ES multimodality therapeutic strategies has resulted from the efforts of several national and international groups in Europe and North America and from cooperation between pediatric and medical oncologists. Successive first-line trials addressed the efficacy of various cyclic combinations of drugs incorporating doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide, and dactinomycin and identified prognostic factors now used to tailor therapies. The role of high-dose chemotherapy is still debated. Current 5-year overall survival for patients with localized disease is 65% to 75%. Patients with metastases have a 5-year overall survival < 30%, except for those with isolated pulmonary metastasis (approximately 50%). Patients with recurrence have a dismal prognosis. The many insights into the biology of the EWS-FLI1 protein in the initiation and progression of ES remain to be translated into novel therapeutic strategies. Current options and future approaches will be discussed. (Less)
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Contribution to journal
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Journal of Clinical Oncology
volume
33
issue
27
pages
140 - 3036
publisher
American Society of Clinical Oncology
external identifiers
  • pmid:26304893
  • wos:000366018800011
  • scopus:84942280113
ISSN
1527-7755
DOI
10.1200/JCO.2014.59.5256
language
English
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yes
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1206a378-ad60-4ce6-bbe7-cc6bff1c59d7 (old id 7834810)
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http://www.ncbi.nlm.nih.gov/pubmed/26304893?dopt=Abstract
date added to LUP
2015-09-07 19:29:17
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2017-11-19 03:12:12
@article{1206a378-ad60-4ce6-bbe7-cc6bff1c59d7,
  abstract     = {Ewing sarcoma (ES) is an aggressive sarcoma of bone and soft tissue occurring at any age with a peak incidence in adolescents and young adults. The treatment of ES relies on a multidisciplinary approach, coupling risk-adapted intensive neoadjuvant and adjuvant chemotherapies with surgery and/or radiotherapy for control of the primary site and possible metastatic disease. The optimization of ES multimodality therapeutic strategies has resulted from the efforts of several national and international groups in Europe and North America and from cooperation between pediatric and medical oncologists. Successive first-line trials addressed the efficacy of various cyclic combinations of drugs incorporating doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide, and dactinomycin and identified prognostic factors now used to tailor therapies. The role of high-dose chemotherapy is still debated. Current 5-year overall survival for patients with localized disease is 65% to 75%. Patients with metastases have a 5-year overall survival &lt; 30%, except for those with isolated pulmonary metastasis (approximately 50%). Patients with recurrence have a dismal prognosis. The many insights into the biology of the EWS-FLI1 protein in the initiation and progression of ES remain to be translated into novel therapeutic strategies. Current options and future approaches will be discussed.},
  author       = {Gaspar, Nathalie and Hawkins, Douglas S and Dirksen, Uta and Lewis, Ian J and Ferrari, Stefano and Le Deley, Marie-Cecile and Kovar, Heinrich and Grimer, Robert and Whelan, Jeremy and Claude, Line and Delattre, Olivier and Paulussen, Michael and Picci, Piero and Sundby Hall, Kirsten and van den Berg, Hendrik and Ladenstein, Ruth and Michon, Jean and Hjorth, Lars and Judson, Ian and Luksch, Roberto and Bernstein, Mark L and Marec-Bérard, Perrine and Brennan, Bernadette and Craft, Alan W and Womer, Richard B and Juergens, Heribert and Oberlin, Odile},
  issn         = {1527-7755},
  language     = {eng},
  number       = {27},
  pages        = {140--3036},
  publisher    = {American Society of Clinical Oncology},
  series       = {Journal of Clinical Oncology},
  title        = {Ewing Sarcoma: Current Management and Future Approaches Through Collaboration.},
  url          = {http://dx.doi.org/10.1200/JCO.2014.59.5256},
  volume       = {33},
  year         = {2015},
}