Current treatment for Ewing's sarcoma

Mihir M. Thacker, H. Thomas Temple, Sean P. Scully

Research output: Contribution to journalArticle

38 Citations (Scopus)

Abstract

Ewing's sarcoma is the second most common primary bone tumor seen in children and adolescents, and was described by James Ewing in 1921 as a diffuse endothelioma of bone. It is one of the differential diagnoses of pediatric small round blue cell tumors. This is not a single condition, but a group of morphologically and clinically closely related disorders with similar molecular biology - expression of tumor-specific chimeric oncoproteins through balanced chromosomal translocations involving the EWS gene - often referred to as the Ewing family of tumors. This includes Ewing's sarcoma of bone, extra-osseous Ewing's sarcoma, Askin tumor and peripheral neuroectodermal tumor. These are aggressive neoplasms with almost 25% of patients having clinically evident metastases at presentation. Ewing's sarcoma has therefore been considered as a systemic disease necessitating local as well as systemic treatment. An aggressive multidisciplinary approach has resulted in significant improvement in prognosis for patients with these tumors. Despite aggressive treatment, 20-40% of patients with localized disease and almost 80% of patients with metastatic disease at presentation succumb to the illness. Advances in understanding the molecular biology of these tumors will hopefully result in the development of novel treatment approaches. The aim of this article is to review the existing treatment methods and to highlight the more recent approaches to the treatment of this condition.

Original languageEnglish
Pages (from-to)319-331
Number of pages13
JournalExpert Review of Anticancer Therapy
Volume5
Issue number2
DOIs
StatePublished - Apr 1 2005

Fingerprint

Ewing's Sarcoma
Neoplasms
Bone and Bones
Molecular Biology
Therapeutics
Peripheral Primitive Neuroectodermal Tumors
Genetic Translocation
Oncogene Proteins
Differential Diagnosis
Pediatrics
Neoplasm Metastasis
Genes

Keywords

  • Chemotherapy
  • Ewing's sarcoma
  • Immunotherapy
  • Limb salvage
  • Pediatric malignancies
  • Radiation therapy
  • Solid tumor
  • Surgery

ASJC Scopus subject areas

  • Pharmacology
  • Cancer Research

Cite this

Thacker, M. M., Thomas Temple, H., & Scully, S. P. (2005). Current treatment for Ewing's sarcoma. Expert Review of Anticancer Therapy, 5(2), 319-331. https://doi.org/10.1586/14737140.5.2.319

Current treatment for Ewing's sarcoma. / Thacker, Mihir M.; Thomas Temple, H.; Scully, Sean P.

In: Expert Review of Anticancer Therapy, Vol. 5, No. 2, 01.04.2005, p. 319-331.

Research output: Contribution to journalArticle

Thacker, MM, Thomas Temple, H & Scully, SP 2005, 'Current treatment for Ewing's sarcoma', Expert Review of Anticancer Therapy, vol. 5, no. 2, pp. 319-331. https://doi.org/10.1586/14737140.5.2.319
Thacker, Mihir M. ; Thomas Temple, H. ; Scully, Sean P. / Current treatment for Ewing's sarcoma. In: Expert Review of Anticancer Therapy. 2005 ; Vol. 5, No. 2. pp. 319-331.
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