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Ewing Sarcoma




The most important prognostic features for Ewing sarcoma (ES) are:

  • The presence/absence of metastatic disease:
    • Metastatic disease has a poor prognosis with only approximately 30% surviving long term. 
    • Localized disease fares much better with current 5 yr EFS of 65-75% depending upon patient age, tumor location and response to chemotherapy.
  • The size, extent and location of primary tumor.
  • Certain cytogenetic abnormalities (including gain of 1q and loss of 16q), and age >14years confer a worse prognosis.


Prognostic Features of ES:





Localized disease



Tumor volume < 200 ml


Peripheral location (distal extremity ES has the best prognosis)


Low serum LDH at diagnosis


No systemic symptoms


Age<12 years



Metastatic disease at presentation


Tumor volume >200 ml


Pelvic location




High serum LDH at diagnosis

Presence of anemia/fever


Age>12 years




Post-chemotherapy necrosis >90%

Post-chemotherapy necrosis <90%

Detectable fusion transcripts in bone marrow No fusion transcript detection in marrow (morphology apparently normal)

Fusion transcript detected in marrow associated with an increased risk of relapse











Chromosome 1p deletion absent


P53 expression absent


Trisomy 8


Complex karyotype:

  • presence of 5 or more independent chromosome abnormalities at diagnosis
  • modal chromosome numbers lower than 50




Chromosome 1p deletion


P53 expression



The following are not thought to be adverse prognostic factors:

  • Presence of a pathological fracture
  • The degree of neural differentiation within the tumor
  • EWS-FL1 translocation is not an adverse prognostic factor






Ewing sarcoma family of tumors at the NCI

Bacci G, Longhi A, Ferrari S, et al.: Prognostic factors in non-metastatic Ewing's sarcoma tumor of bone: an analysis of 579 patients treated at a single institution with adjuvant or neoadjuvant chemotherapy between 1972 and 1998. Acta Oncol 45 (4): 469-75, 2006

Rodríguez-Galindo C, Liu T, Krasin MJ, et al.: Analysis of prognostic factors in ewing sarcoma family of tumors: review of St. Jude Children's Research Hospital studies. Cancer 110 (2): 375-84, 2007

Schleiermacher G, Peter M, Oberlin O, et al.: Increased risk of systemic relapses associated with bone marrow micrometastasis and circulating tumor cells in localized ewing tumor. J Clin Oncol 21 (1): 85-91, 2003.

Roberts P, Burchill SA, Brownhill S, et al.: Ploidy and karyotype complexity are powerful prognostic indicators in the Ewing's sarcoma family of tumors: a study by the United Kingdom Cancer Cytogenetics and the Children's Cancer and Leukaemia Group. Genes Chromosomes Cancer 47 (3): 207-20, 2008.


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