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

      1 , 1 , 1 , 2 , 3 , 4 , 6 , 7 , 8 , 9 , 10 , 1 , 11 , 14 , 8 , 15 , 1 , 3 , 16 , 14 , 14 , 17 , 18 , 11 , 19 , 10 , 20 , 10 , 21 , 3 , 22 , 11 , 2 , 5 , 4 , 23 , 24 , , 8 , 12 , 3 , 10 , 25 , 11 , 1 , The PanCareSurFup Consortium

      JNCI Journal of the National Cancer Institute

      Oxford University Press

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          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.

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          Most cited references 22

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          Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases.

          A review was done of 120 cases of malignant peripheral nerve sheath tumor (MPNST) seen during a 71-year period. Of the 120 patients, 52 were males and 68 were females with a mean age at diagnosis of 35.3 years; 12 patients were younger than 20 years. The series included 62 (52%) patients with neurofibromatosis, 13 (11%) with postradiation sarcomas, and 19 (16%) with metaplastic foci. The incidence of MPNST arising in neurofibromatosis was 4.6% in the current series and 0.001% in the general clinic population. Tumors greater than 5 cm and the presence of neurofibromatosis adversely affected the prognosis (P less than 0.05). When both features were present, survival was greatly decreased. Patients with tumor in the extremities did better than those with head or neck lesions. Metaplastic foci or previous radiation at the tumor site did not alter the prognosis. Each tumor was graded 1 to 4 on the basis of cellularity, pleomorphism, mitotic index, and necrosis. No significant correlation was noted between survival and either grade or mitotic rate. Survival was improved when total rather than subtotal resection was done. This was most marked in patients with a small lesion, which may reflect the difficulty in adequately excising large tumors. Adjuvant radiation or chemotherapy did not appear to affect survival. The MPNST is an aggressive uncommon neoplasm, and large tumor size, the presence of neurofibromatosis, and total resection are the most important prognostic indicators.
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            Subsequent neoplasms in 5-year survivors of childhood cancer: the Childhood Cancer Survivor Study.

            The occurrence of subsequent neoplasms has direct impact on the quantity and quality of life in cancer survivors. We have expanded our analysis of these events in the Childhood Cancer Survivor Study (CCSS) to better understand the occurrence of these events as the survivor population ages. The incidence of and risk for subsequent neoplasms occurring 5 years or more after the childhood cancer diagnosis were determined among 14,359 5-year survivors in the CCSS who were treated from 1970 through 1986 and who were at a median age of 30 years (range = 5-56 years) for this analysis. At 30 years after childhood cancer diagnosis, we calculated cumulative incidence at 30 years of subsequent neoplasms and calculated standardized incidence ratios (SIRs), excess absolute risks (EARs) for invasive second malignant neoplasms, and relative risks for subsequent neoplasms by use of multivariable Poisson regression. Among 14,359 5-year survivors, 1402 subsequently developed 2703 neoplasms. Cumulative incidence at 30 years after the childhood cancer diagnosis was 20.5% (95% confidence interval [CI] = 19.1% to 21.8%) for all subsequent neoplasms, 7.9% (95% CI = 7.2% to 8.5%) for second malignant neoplasms (excluding nonmelanoma skin cancer), 9.1% (95% CI = 8.1% to 10.1%) for nonmelanoma skin cancer, and 3.1% (95% CI = 2.5% to 3.8%) for meningioma. Excess risk was evident for all primary diagnoses (EAR = 2.6 per 1000 person-years, 95% CI = 2.4 to 2.9 per 1000 person-years; SIR = 6.0, 95% CI = 5.5 to 6.4), with the highest being for Hodgkin lymphoma (SIR = 8.7, 95% CI = 7.7 to 9.8) and Ewing sarcoma (SIR = 8.5, 95% CI = 6.2 to 11.7). In the Poisson multivariable analysis, female sex, older age at diagnosis, earlier treatment era, diagnosis of Hodgkin lymphoma, and treatment with radiation therapy were associated with increased risk of subsequent neoplasm. As childhood cancer survivors progress through adulthood, risk of subsequent neoplasms increases. Patients surviving Hodgkin lymphoma are at greatest risk. There is no evidence of risk reduction with increasing duration of follow-up.
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              Long-term risks of subsequent primary neoplasms among survivors of childhood cancer.

              Survivors of childhood cancer are at excess risk of developing subsequent primary neoplasms but the long-term risks are uncertain. To investigate long-term risks of subsequent primary neoplasms in survivors of childhood cancer, to identify the types that contribute most to long-term excess risk, and to identify subgroups of survivors at substantially increased risk of particular subsequent primary neoplasms that may require specific interventions. British Childhood Cancer Survivor Study--a population-based cohort of 17,981 5-year survivors of childhood cancer diagnosed with cancer at younger than 15 years between 1940 and 1991 in Great Britain, followed up through December 2006. Standardized incidence ratios (SIRs), absolute excess risks (AERs), and cumulative incidence of subsequent primary neoplasms. After a median follow-up time of 24.3 years (mean = 25.6 years), 1354 subsequent primary neoplasms were ascertained; the most frequently observed being central nervous system (n = 344), nonmelanoma skin cancer (n = 278), digestive (n = 105), genitourinary (n = 100), breast (n = 97), and bone (n = 94). The overall SIR was 4 times more than expected (SIR, 3.9; 95% confidence interval [CI], 3.6-4.2; AER, 16.8 per 10,000 person-years). The AER at older than 40 years was highest for digestive and genitourinary subsequent primary neoplasms (AER, 5.9 [95% CI, 2.5-9.3]; and AER, 6.0 [95%CI, 2.3-9.6] per 10,000 person-years, respectively); 36% of the total AER was attributable to these 2 subsequent primary neoplasm sites. The cumulative incidence of colorectal cancer for survivors treated with direct abdominopelvic irradiation was 1.4% (95% CI, 0.7%-2.6%) by age 50 years, comparable with the 1.2% risk in individuals with at least 2 first-degree relatives affected by colorectal cancer. Among a cohort of British childhood cancer survivors, the greatest excess risk associated with subsequent primary neoplasms at older than 40 years was for digestive and genitourinary neoplasms.
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                Author and article information

                Journal
                J Natl Cancer Inst
                J. Natl. Cancer Inst
                jnci
                JNCI Journal of the National Cancer Institute
                Oxford University Press
                0027-8874
                1460-2105
                June 2018
                20 November 2017
                20 November 2017
                : 110
                : 6
                : 649-660
                Affiliations
                [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 ]Hungarian Childhood Cancer Registry, Semmelweis University, Budapest, Hungary
                [6 ]2nd Department of Pediatrics, Semmelweis University, Budapest, Hungary
                [7 ]Kepler Universitätsklinikum, Linz, Austria
                [8 ]Danish Cancer Society Research Center, Survivorship Unit, Copenhagen, Denmark
                [9 ]Boyne Research Institute, Drogheda, Ireland
                [10 ]Department of Pediatric Oncology, Emma Children’s Hospital/Academic Medical Center, Amsterdam, the Netherlands
                [11 ]Lund University, Skane University Hospital, Department of Clinical Sciences Lund, Pediatrics, Lund, Sweden
                [12 ]Department of Clinical Medicine, Faculty of Health, Aarhus University, Aarhus, Denmark
                [14 ]German Childhood Cancer Registry (GCCR), Institute of Medical Biostatistics, Epidemiology and Informatics, University Medical Center, Mainz, Germany
                [15 ]Childreńs Hospital, Landspitali University Hospital, Reykjavik, Iceland
                [16 ]Foundation MBBM, Hemato-Oncology Center, University of Milano-Bicocca, Monza, Italy
                [17 ]Swiss Childhood Cancer Registry, Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland
                [18 ]Department of Paediatrics, University Children's Hospital of Bern, University of Bern, Bern, Switzerland
                [19 ]Norwegian National Advisory Unit on Solid Tumors in Children, Oslo, Norway
                [20 ]Great North Children's Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, and Northern Institute of Cancer Research, Newcastle University, Newcastle upon Tyne, UK
                [21 ]Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Italy
                [22 ]Norwegian Cancer Registry and Department of Pediatric Medicine, Oslo University Hospital and Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Norway
                [23 ]Department of Pediatric and Adolescent Medicine, Turku University and Turku University Hospital, Turku, Finland
                [24 ]Institute of Oncology, Ljubljana, Slovenia
                [25 ]Department of Pediatric Oncology, Princess Maxima Center for Pediatric Oncology, Utrecht, the Netherlands
                Author notes

                See the Notes section for the full list of authors and affiliations.

                Correspondence to: Raoul C. Reulen, PhD, Centre for Childhood Cancer Survivor Studies, Institute of Applied Health Research, Robert Aitken Building, University of Birmingham, Birmingham, United Kingdom B15 2TT (e-mail: r.c.reulen@ 123456bham.ac.uk ).
                Article
                djx235
                10.1093/jnci/djx235
                6005019
                29165710
                © The Author 2017. Published by Oxford University Press.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

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                Pages: 12
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