Sarcomasdown arrow

Rhabdomyosarcoma

Non-RMS

 

 

Ewing Sarcoma

 

 

Prognosis

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:

 

FAVORABLE

UNFAVORABLE

Clinical

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

 

Female

Metastatic disease at presentation

 

Tumor volume >200 ml

 

Pelvic location

 

 

 

High serum LDH at diagnosis

Presence of anemia/fever

 

Age>12 years

 

Male

Pathological

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

Genetic

 

 

 

 

 

 

 

Diploid

 

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

 

Aneuploidy

 

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

 

 

 

 

References:

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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