Ewing's sarcoma family of tumors: Current management

Mark Bernstein, Heinrich Kovar, Michael Paulussen, R Randall, Andreas Schuck, Lisa A. Teot, Herbert Juergens

Research output: Contribution to journalArticle

282 Citations (Scopus)

Abstract

Ewing's sarcoma is the second most frequent primary bone cancer, with approximately 225 new cases diagnosed each year in patients less than 20 years of age in North America. It is one of the pediatric small round blue cell tumors, characterized by strong membrane expression of CD99 in a chain-mail pattern and negativity for lymphoid (CD45), rhabdomyosarcoma (myogenin, desmin, actin) and neuroblastoma (neurofilament protein) markers. Pathognomonic translocations involving the ews gene on chromosome 22 and an ets-type gene, most commonly the fli1 gene on chromosome 11, are implicated in the great majority of cases. Clinical presentation is usually dominated by local bone pain and a mass. Imaging reveals a technetium pyrophosphate avid lesion that, on plain radiograph, is destructive, diaphyseal and classically causes layered periosteal calcification. Magnetic resonance best defines the extent of the lesion. Biopsy should be undertaken by an experienced orthopedic oncologist. Approximately three quarters of patients have initially localized disease. About two thirds survive disease-free. Management, preferably at a specialist center with a multi-disciplinary team, includes both local control - either surgery, radiation or a combination - and systemic chemotherapy. Chemotherapy includes cyclic combinations, incorporating vincristine, doxorubicin, cyclophosphamide, etoposide, ifosfamide and occasionally actinomycin D. Topotecan in combination with cyclophosphamide has shown preliminary activity. Patients with initially metastatic disease fare less well, with about one quarter surviving. Studies incorporating intensive therapy followed by stem cell infusion show no clear benefit. New approaches include anti-angiogenic therapy, particularly since vascular endothelial growth factor is an apparent downstream target of the ews-fli1 oncogene.

Original languageEnglish (US)
Pages (from-to)503-519
Number of pages17
JournalOncologist
Volume11
Issue number5
DOIs
StatePublished - May 1 2006
Externally publishedYes

Fingerprint

Ewing's Sarcoma
Cyclophosphamide
Myogenin
Genes
Topotecan
Neurofilament Proteins
Chromosomes, Human, Pair 22
Bone Neoplasms
Neoplasms
Ifosfamide
Chromosomes, Human, Pair 11
Desmin
Rhabdomyosarcoma
Technetium
Postal Service
Dactinomycin
Vincristine
Etoposide
North America
Combination Drug Therapy

Keywords

  • Adolescents and young adults
  • Bone cancer
  • Ewing's sarcoma
  • Multimodal therapy
  • Pediatrics

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

Bernstein, M., Kovar, H., Paulussen, M., Randall, R., Schuck, A., Teot, L. A., & Juergens, H. (2006). Ewing's sarcoma family of tumors: Current management. Oncologist, 11(5), 503-519. https://doi.org/10.1634/theoncologist.11-5-503

Ewing's sarcoma family of tumors : Current management. / Bernstein, Mark; Kovar, Heinrich; Paulussen, Michael; Randall, R; Schuck, Andreas; Teot, Lisa A.; Juergens, Herbert.

In: Oncologist, Vol. 11, No. 5, 01.05.2006, p. 503-519.

Research output: Contribution to journalArticle

Bernstein, M, Kovar, H, Paulussen, M, Randall, R, Schuck, A, Teot, LA & Juergens, H 2006, 'Ewing's sarcoma family of tumors: Current management', Oncologist, vol. 11, no. 5, pp. 503-519. https://doi.org/10.1634/theoncologist.11-5-503
Bernstein M, Kovar H, Paulussen M, Randall R, Schuck A, Teot LA et al. Ewing's sarcoma family of tumors: Current management. Oncologist. 2006 May 1;11(5):503-519. https://doi.org/10.1634/theoncologist.11-5-503
Bernstein, Mark ; Kovar, Heinrich ; Paulussen, Michael ; Randall, R ; Schuck, Andreas ; Teot, Lisa A. ; Juergens, Herbert. / Ewing's sarcoma family of tumors : Current management. In: Oncologist. 2006 ; Vol. 11, No. 5. pp. 503-519.
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