Cancer predisposition syndromes in children
The aim of the study was to assess the prevalence of cancer predisposition syndromes among children with cancer in Moscow Region (MR). The data on patients were retrieved from the database of Childhood Population-based Cancer Registry of MR. 35 (3.0 %) children with cancer predisposition syndromes...
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| Format: | Article |
| Language: | Russian |
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ABV-press
2022-11-01
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| Series: | Онкогематология |
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| Online Access: | https://oncohematology.abvpress.ru/ongm/article/view/743 |
| _version_ | 1826557065585229824 |
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| author | D. Yu. Kachanov R. T. Abdullaev T. V. Shamanskaya Ye. V. Inyushkina K. V. Dobrenkov R. F. Savkova S. R. Varfolomeeva |
| author_facet | D. Yu. Kachanov R. T. Abdullaev T. V. Shamanskaya Ye. V. Inyushkina K. V. Dobrenkov R. F. Savkova S. R. Varfolomeeva |
| author_sort | D. Yu. Kachanov |
| collection | DOAJ |
| description | The aim of the study was to assess the prevalence of cancer predisposition syndromes among children with cancer in Moscow Region (MR). The data on patients were retrieved from the database of Childhood Population-based Cancer Registry of MR. 35 (3.0 %) children with cancer predisposition syndromes were revealed among 1173 registered from 2000 till February 2010. The most prevalent syndromes were hereditary retinoblastoma (RB) – 9 (25.7 %), Down syndrome – 8 (22.8 %) and neurofibromatosis type 1–8 (22.8 %). Genetic syndromes were observed in patients with retinoblastoma – 31 %, germ-cell tumors – 6.8 % and soft tissue sarcomas (STS) – 4.5 %. The increased risk of development of leukemia in patients with Down syndrome was observed. The relative risk (RR) of 15.0 (95 % CI 1.9–333.1) for leukemia and 57.1 (95 % CI 9.3–1398.0) for AML was observed. The association of neurofibromatosis type 1 with the development of tumors of central nervous system and soft tissue sarcomas was proved. The RR was 62.5 (95 % CI 8.1–1388.6) for CNS tumors and 150,0 (95 % CI 26.9–3480.9) for STS. |
| first_indexed | 2024-04-09T20:23:51Z |
| format | Article |
| id | doaj.art-02d9d9d97f244888a36e09149ac62ccc |
| institution | Directory Open Access Journal |
| issn | 1818-8346 2413-4023 |
| language | Russian |
| last_indexed | 2025-03-14T08:22:40Z |
| publishDate | 2022-11-01 |
| publisher | ABV-press |
| record_format | Article |
| series | Онкогематология |
| spelling | doaj.art-02d9d9d97f244888a36e09149ac62ccc2025-03-02T13:07:15ZrusABV-pressОнкогематология1818-83462413-40232022-11-01032935621Cancer predisposition syndromes in childrenD. Yu. Kachanov0R. T. Abdullaev1T. V. Shamanskaya2Ye. V. Inyushkina3K. V. Dobrenkov4R. F. Savkova5S. R. Varfolomeeva6Federal research center of pediatric hematology, oncology and immunology; Moscow Regional Oncological HospitalFederal research center of pediatric hematology, oncology and immunology; Scientific and Applied Center for the Treatment of Children with Craniofacial and Neurological DisordersFederal research center of pediatric hematology, oncology and immunologyFederal research center of pediatric hematology, oncology and immunology; Moscow Regional Oncological HospitalFederal research center of pediatric hematology, oncology and immunologyMoscow Regional Oncological HospitalFederal research center of pediatric hematology, oncology and immunologyThe aim of the study was to assess the prevalence of cancer predisposition syndromes among children with cancer in Moscow Region (MR). The data on patients were retrieved from the database of Childhood Population-based Cancer Registry of MR. 35 (3.0 %) children with cancer predisposition syndromes were revealed among 1173 registered from 2000 till February 2010. The most prevalent syndromes were hereditary retinoblastoma (RB) – 9 (25.7 %), Down syndrome – 8 (22.8 %) and neurofibromatosis type 1–8 (22.8 %). Genetic syndromes were observed in patients with retinoblastoma – 31 %, germ-cell tumors – 6.8 % and soft tissue sarcomas (STS) – 4.5 %. The increased risk of development of leukemia in patients with Down syndrome was observed. The relative risk (RR) of 15.0 (95 % CI 1.9–333.1) for leukemia and 57.1 (95 % CI 9.3–1398.0) for AML was observed. The association of neurofibromatosis type 1 with the development of tumors of central nervous system and soft tissue sarcomas was proved. The RR was 62.5 (95 % CI 8.1–1388.6) for CNS tumors and 150,0 (95 % CI 26.9–3480.9) for STS.https://oncohematology.abvpress.ru/ongm/article/view/743childrencancercancer predisposition syndromescancer registry |
| spellingShingle | D. Yu. Kachanov R. T. Abdullaev T. V. Shamanskaya Ye. V. Inyushkina K. V. Dobrenkov R. F. Savkova S. R. Varfolomeeva Cancer predisposition syndromes in children Онкогематология children cancer cancer predisposition syndromes cancer registry |
| title | Cancer predisposition syndromes in children |
| title_full | Cancer predisposition syndromes in children |
| title_fullStr | Cancer predisposition syndromes in children |
| title_full_unstemmed | Cancer predisposition syndromes in children |
| title_short | Cancer predisposition syndromes in children |
| title_sort | cancer predisposition syndromes in children |
| topic | children cancer cancer predisposition syndromes cancer registry |
| url | https://oncohematology.abvpress.ru/ongm/article/view/743 |
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