Cancer predisposition syndromes in children

D. Yu. Kachanov; Качанов Д. Ю.; R. T. Abdullaev; Абдуллаев Р. Т.; T. V. Shamanskaya; Шаманская Т. В.; Ye. V. Inyushkina; Инюшкина Е. В.; K. V. Dobrenkov; Добреньков К. В.; R. F. Savkova; Савкова Р. Ф.; S. R. Varfolomeeva; Варфоломеева С. Р.

Cancer predisposition syndromes in children

Authors: Kachanov D.Y.¹^,2, Abdullaev R.T.¹^,3, Shamanskaya T.V.¹, Inyushkina Y.V.¹^,2, Dobrenkov K.V.¹, Savkova R.F.², Varfolomeeva S.R.¹
Affiliations:
1. Federal research center of pediatric hematology, oncology and immunology
2. Moscow Regional Oncological Hospital
3. Scientific and Applied Center for the Treatment of Children with Craniofacial and Neurological Disorders
Issue: No 3 (2010)
Pages: 29-35
Section: HEMATOLOGIC MALIGNANCIES: DIAGNOSIS, TREATMENT, SUPPORTIVE CARE
Published: 26.08.2010
URL: https://oncohematology.abvpress.ru/ongm/article/view/743
ID: 743

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Abstract

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.

Keywords

children, cancer, cancer predisposition syndromes, cancer registry

About the authors

D. Yu. Kachanov

Federal research center of pediatric hematology, oncology and immunology; Moscow Regional Oncological Hospital

Author for correspondence.
Email: totti111@list.ru

Moscow

Balashiha

Russian Federation

R. T. Abdullaev

Federal research center of pediatric hematology, oncology and immunology; Scientific and Applied Center for the Treatment of Children with Craniofacial and Neurological Disorders

Moscow

Russian Federation