Review Article

Ewing's Sarcoma and Second Malignancies

Table 1


Author (population studied)Median FollowupTreatment yearsTotal patient (#)SMN
(#)
SMN typesSignificant Risks for SMN5/10/15/20 year SMN ratesRisk calculation

Kuttesch, et al. (NCI, SJCRH, Florida)9.5 yearsPrior to 19902661614 solid tumor
2 hematologic
Treating institution, Any RT, Higher sarcoma risk with higher RT ( 60 Gray) doseNR
5%
NR
9.2%
AR = 33.8/10 K PY for sarcoma AR = 54.7/10 K PY for all SMN

Fuchs, et al. (Mayo)7.4 years1975–19993972921 solid tumor
8 hematologic
NR6.5% at 7.4 yearsNR

Dunst, et al. (CESS)5.1 years1981–199167483 solid tumor
5 hematologic
None significant, Trend toward increased solid SMN with any RT0.7%
2.9%
4.7%
NR
NR

Bacci, et al. (Rizzoli)Range of 5–33 years1972–19995971411 solid tumor
3 hematologic
Higher for “full dose” RT versus post-op “reduced dose”3%
6.5%
NR
12.7%
NR

Ginsberg, et al. (CCSS)23 years (mean)1970–19864033634 solid tumor
2 hematologic
NR9% at 20 yearsSIR = 5.9 AR = 48.1/10 K PY

Bhatia, et al. (CCG/POG)8.03 years1988-1992587164 solid tumor
12 hematologic
Treatment arm CNRSIR = 127.7 for myeloid SMN

Paulussen, et al. (EICESS)4.6 years1992-199969062 solid tumor
4 hematologic
None significant, Trend toward increased risk after etoposide0.93%
NR
NR
NR
SIR = 30.9–33.4 for hematologic SMN (compared to Saarland & SEER registries)

Navid, et al. (SJCRH)12.2 years1979–2004237124 solid tumor
8 hematologic
More recent treatment protocols, Lower stage disease3.0%
4.7%
NR
NR
SIR = 17.8 overall
SIR = 65.1 on more recent protocols

Sultan, et al. (SEER) 6.7 years1973–200511663523 solid tumor
12 hematologic
Treatment era (1973–85), Any radiation (OR = 2.55)2.1%
4.4%
NR
8.0%
O/E =  4.01
O/E = 51.09 for myeloid SMN
O/E = 51.08 for OS

Abbreviations: SMN: second malignant neoplasm, NCI: National Cancer Institute, SJCRH: St Jude Children’s Research Hospital, CESS: Cooperative Ewing’s Sarcoma Study, CCG: Children’s Cancer Group, POG: Pediatric Oncology Group, EICESS: European Intergroup Cooperative Ewing’s Sarcoma Study, SEER: Surveillance, Epidemiology and End Results, RT: radiation therapy, NR: not reported, AR: absolute risk, PY: person years, OR: odds ratio, SIR: standardized incidence ratio, O/E ratio of observed to expected cases OS: osteosarcoma