OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press743HEMATOLOGIC MALIGNANCIES: DIAGNOSIS, TREATMENT, SUPPORTIVE CAREГЕМОБЛАСТОЗЫ: ДИАГНОСТИКА, ЛЕЧЕНИЕ, СОПРОВОДИТЕЛЬНАЯ ТЕРАПИЯCancer predisposition syndromes in childrenГенетические синдромы у детей со злокачественными новообразованиямиKachanovD. Yu.КачановД. Ю.
Russian Federation

Moscow

Balashiha

Денис Юрьевич Качанов

ФГУ Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии

Москва

Балашиха

totti111@list.ruAbdullaevR. T.АбдуллаевР. Т.
Russian Federation

Moscow

ФГУ Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии

Москва

ShamanskayaT. V.ШаманскаяТ. В.
Russian Federation

Moscow

ФГУ Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии

Москва

InyushkinaYe. V.ИнюшкинаЕ. В.
Russian Federation

Moscow

Balashiha

ФГУ Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии

Москва

Балашиха

DobrenkovK. V.ДобреньковК. В.
Russian Federation

Moscow

ФГУ Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии

Москва

SavkovaR. F.СавковаР. Ф.
Russian Federation

Balashiha

Балашиха

VarfolomeevaS. R.ВарфоломееваС. Р.
Russian Federation

Moscow

ФГУ Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии

Москва

Federal research center of pediatric hematology, oncology and immunologyМинздравсоцразвития РоссииMoscow Regional Oncological HospitalМосковский областной онкологический диспансерScientific and Applied Center for the Treatment of Children with Craniofacial and Neurological DisordersНаучно-практический центр медицинской помощи детям с пороками развития челюстно-лицевой области и врожденными заболеваниями нервной системы260820103293525112022251120222010https://oncohematology.abvpress.ru/ongm/article/view/743

   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.

   Цель настоящего исследования – изучение распространенности генетических синдромов у детей со злокачественными новообразованиями (ЗН) на территории Московской области (МО).

   Данные о пациентах были получены из базы данных Детского популяционного канцер-регистра МО. Всего за период с января 2000 по февраль 2010 гг. на территории МО зарегистрировано 1173 случая заболевания детей ЗН, из них генетические синдромы выявлены у 35 (3,0 %) пациентов. В 9 (25,7 %) случаях имела место наследственная ретинобластома, по 8 (22,8 %) случаев пришлось на синдром Дауна и нейрофиброматоз I типа. Наиболее часто генетические синдромы выявлялись у больных с ретинобластомой – 31 %, герминогенными опухолями – 6,8 % и саркомами мягких тканей – 4,5 %. Показан повышенный риск развития лейкозов у детей с синдромом Дауна. Относительный риск (ОР) развития лейкозов и острого миелобластного лейкоза (ОМЛ) составил 15,0 (95 % ДИ = 1,9–333,1) и 57,1 (95 % ДИ = 9,3–1398,0) соответственно. Подтверждена ассоциация нейрофиброматоза I типа с развитием опухолей центральной нервной системы (ЦНС) и сарком мягких тканей. ОР составил 62,5 (95 % ДИ = 8,1–1388,6) и 150,0 (95 % ДИ = 26,9–3480,9) соответственно.

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