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
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:
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
