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J Natl Cancer Inst. 2018 Jun 1;110(6):649-660. doi: 10.1093/jnci/djx235.

Risk of Soft-Tissue Sarcoma Among 69 460 Five-Year Survivors of Childhood Cancer in Europe.

Author information

1
Center for Childhood Cancer Survivor Studies, Institute of Applied Health Research, Robert Aitken Building, University of Birmingham, Birmingham, UK.
2
Childhood Cancer Registry of Piedmont, Cancer Epidemiology Unit, Department of Medical Sciences, University of Turin and AOU Città della Salute e della Scienza di Torino, Torino, Italy.
3
Cancer and Radiation Team, U1018 INSERM, Gustave Roussy, Villejuif, France.
4
Epidemiology and Biostatistics Section, Gaslini Children Hospital, Genova, Italy.
5
2nd Department of Pediatrics, Semmelweis University, Budapest, Hungary.
6
Kepler Universitätsklinikum, Linz, Austria.
7
Danish Cancer Society Research Center, Survivorship Unit, Copenhagen, Denmark.
8
Boyne Research Institute, Drogheda, Ireland.
9
Department of Pediatric Oncology, Emma Children's Hospital/Academic Medical Center, Amsterdam, the Netherlands.
10
Lund University, Skane University Hospital, Department of Clinical Sciences Lund, Pediatrics, Lund, Sweden.
11
German Childhood Cancer Registry (GCCR), Institute of Medical Biostatistics, Epidemiology and Informatics, University Medical Center, Mainz, Germany.
12
Childreńs Hospital, Landspitali University Hospital, Reykjavik, Iceland.
13
Foundation MBBM, Hemato-Oncology Center, University of Milano-Bicocca, Monza, Italy.
14
Swiss Childhood Cancer Registry, Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland.
15
Department of Paediatrics, University Children's Hospital of Bern, University of Bern, Bern, Switzerland.
16
Norwegian National Advisory Unit on Solid Tumors in Children, Oslo, Norway.
17
Great North Children's Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, and Northern Institute of Cancer Research, Newcastle University, Newcastle upon Tyne, UK.
18
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy.
19
Norwegian Cancer Registry and Department of Pediatric Medicine, Oslo University Hospital and Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Norway.
20
Hungarian Childhood Cancer Registry, Semmelweis University, Budapest, Hungary.
21
Department of Pediatric and Adolescent Medicine, Turku University and Turku University Hospital, Turku, Finland.
22
Institute of Oncology, Ljubljana, Slovenia.
23
Department of Clinical Medicine, Faculty of Health, Aarhus University, Aarhus, Denmark.
24
Department of Pediatric Oncology, Princess Maxima Center for Pediatric Oncology, Utrecht, the Netherlands.

Abstract

Background:

Childhood cancer survivors are at risk of subsequent primary soft-tissue sarcomas (STS), but the risks of specific STS histological subtypes are unknown. We quantified the risk of STS histological subtypes after specific types of childhood cancer.

Methods:

We pooled data from 13 European cohorts, yielding a cohort of 69 460 five-year survivors of childhood cancer. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) were calculated.

Results:

Overall, 301 STS developed compared with 19 expected (SIR = 15.7, 95% confidence interval [CI] = 14.0 to 17.6). The highest standardized incidence ratios were for malignant peripheral nerve sheath tumors (MPNST; SIR = 40.6, 95% CI = 29.6 to 54.3), leiomyosarcomas (SIR = 29.9, 95% CI = 23.7 to 37.2), and fibromatous neoplasms (SIR = 12.3, 95% CI = 9.3 to 16.0). SIRs for MPNST were highest following central nervous system tumors (SIR = 80.5, 95% CI = 48.4 to 125.7), Hodgkin lymphoma (SIR = 81.3, 95% CI = 35.1 to 160.1), and Wilms tumor (SIR = 76.0, 95% CI = 27.9 to 165.4). Standardized incidence ratios for leiomyosarcoma were highest following retinoblastoma (SIR = 342.9, 95% CI = 245.0 to 466.9) and Wilms tumor (SIR = 74.2, 95% CI = 37.1 to 132.8). AERs for all STS subtypes were generally low at all years from diagnosis (AER < 1 per 10 000 person-years), except for leiomyosarcoma following retinoblastoma, for which the AER reached 52.7 (95% CI = 20.0 to 85.5) per 10 000 person-years among patients who had survived at least 45 years from diagnosis of retinoblastoma.

Conclusions:

For the first time, we provide risk estimates of specific STS subtypes following childhood cancers and give evidence that risks of MPNSTs, leiomyosarcomas, and fibromatous neoplasms are particularly increased. While the multiplicative excess risks relative to the general population are substantial, the absolute excess risk of developing any STS subtype is low, except for leiomyosarcoma after retinoblastoma. These results are likely to be informative for both survivors and health care providers.

PMID:
29165710
PMCID:
PMC6005019
DOI:
10.1093/jnci/djx235
[Indexed for MEDLINE]
Free PMC Article

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