Diagnosis and prognosis for the Ewing family of tumors

Emanuela Palmerini, Eric L. Staals, Stefano Ferrari, Gaetano Bacci

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Ewing's sarcoma was first described by James Ewing in 1921. It is the second most common bone sarcoma seen in children, adolescents and young adults after osteosarcoma and belongs to the group of 'small round blue cell tumors', showing an aggressive natural history. Once almost invariably fatal and treated only with palliative radiation, thanks to a multidisciplinary approach, the probability of survival at 5years is now - 65 - 75% for patients with localized disease. This percentage is no more than 20 - 25% for patients with metastatic disease at presentation. Objective: To review epidemiology, diagnosis and prognosis of Ewing's sarcoma family tumors (EFT) of bone. This entity includes a wide spectrum of tumors, from the less differentiated or classic Ewing's sarcoma to the more differentiated peripheral neuroectodermal tumor. A translocation involving the EWS gene on the chromosome 22 band q12 is characteristic of EFTs. Conclusion: A complete clinical and radiological assessment is essential for initial patient evaluation. Owing to the rarity of this entity, whenever an EFT is suspected patients should be referred to a bone tumor treatment center before a diagnostic biopsy is performed. The molecular characterization of chromosomal translocations has an important role in the diagnosis of EFT.

Original languageEnglish
Pages (from-to)445-452
Number of pages8
JournalExpert Opinion on Medical Diagnostics
Volume3
Issue number4
DOIs
Publication statusPublished - Jul 2009

Keywords

  • Bone tumor
  • Diagnosis
  • Ewing's sarcoma
  • Peripheral neuroectodermal tumor

ASJC Scopus subject areas

  • Molecular Medicine
  • Biomedical Engineering
  • Medicine(all)
  • Biochemistry, medical

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