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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="other" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Pediatric Hematology/Oncology and Immunopathology</journal-id><journal-title-group><journal-title xml:lang="en">Pediatric Hematology/Oncology and Immunopathology</journal-title><trans-title-group xml:lang="ru"><trans-title>Вопросы гематологии/онкологии и иммунопатологии в педиатрии</trans-title></trans-title-group></journal-title-group><issn publication-format="print">1726-1708</issn><issn publication-format="electronic">2414-9314</issn><publisher><publisher-name xml:lang="en">Fund Doctors, Innovations, Science for Children</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">606</article-id><article-id pub-id-type="doi">10.24287/1726-1708-2022-21-4-31-44</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>ORIGINAL ARTICLES</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>ОРИГИНАЛЬНЫЕ СТАТЬИ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Effectiveness of cisplatin monotherapy in standard-risk hepatoblastoma</article-title><trans-title-group xml:lang="ru"><trans-title>Эффективность монотерапии цисплатином у пациентов с гепатобластомой группы стандартного риска</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9654-084X</contrib-id><name-alternatives><name xml:lang="en"><surname>Moiseenko</surname><given-names>R. A.</given-names></name><name xml:lang="ru"><surname>Моисеенко</surname><given-names>Р. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1387-209X</contrib-id><name-alternatives><name xml:lang="en"><surname>Akhaladze</surname><given-names>D. G.</given-names></name><name xml:lang="ru"><surname>Ахаладзе</surname><given-names>Д. Г.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4205-5627</contrib-id><name-alternatives><name xml:lang="en"><surname>Filin</surname><given-names>A. V.</given-names></name><name xml:lang="ru"><surname>Филин</surname><given-names>А. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-1806-9180</contrib-id><name-alternatives><name xml:lang="en"><surname>Kim</surname><given-names>E. F.</given-names></name><name xml:lang="ru"><surname>Ким</surname><given-names>Э. Ф.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Krasnogorsk</italic></p></bio><bio xml:lang="ru"><p><italic>Красногорск</italic></p></bio><xref ref-type="aff" rid="aff3"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5691-2522</contrib-id><name-alternatives><name xml:lang="en"><surname>Rabaev</surname><given-names>G. S.</given-names></name><name xml:lang="ru"><surname>Рабаев</surname><given-names>Г. С.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2348-221X</contrib-id><name-alternatives><name xml:lang="en"><surname>Feoktistova</surname><given-names>E. V.</given-names></name><name xml:lang="ru"><surname>Феоктистова</surname><given-names>Е. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff4"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0404-6420</contrib-id><name-alternatives><name xml:lang="en"><surname>Merkulov</surname><given-names>N. N.</given-names></name><name xml:lang="ru"><surname>Меркулов</surname><given-names>Н. Н.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2399-6811</contrib-id><name-alternatives><name xml:lang="en"><surname>Metelin</surname><given-names>A. V.</given-names></name><name xml:lang="ru"><surname>Метелин</surname><given-names>А. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7317-7104</contrib-id><name-alternatives><name xml:lang="en"><surname>Tereshchenko</surname><given-names>G. V.</given-names></name><name xml:lang="ru"><surname>Терещенко</surname><given-names>Г. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-7945-2565</contrib-id><name-alternatives><name xml:lang="en"><surname>Shchukin</surname><given-names>V. V.</given-names></name><name xml:lang="ru"><surname>Щукин</surname><given-names>В. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3767-4477</contrib-id><name-alternatives><name xml:lang="en"><surname>Shamanskaya</surname><given-names>T. V.</given-names></name><name xml:lang="ru"><surname>Шаманская</surname><given-names>Т. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6131-1783</contrib-id><name-alternatives><name xml:lang="en"><surname>Varfolomeeva</surname><given-names>S. R.</given-names></name><name xml:lang="ru"><surname>Варфоломеева</surname><given-names>С. Р.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>Moscow</italic></p></bio><bio xml:lang="ru"><p><italic>Москва</italic></p></bio><xref ref-type="aff" rid="aff5"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3704-8783</contrib-id><name-alternatives><name xml:lang="en"><surname>Kachanov</surname><given-names>D. Yu.</given-names></name><name xml:lang="ru"><surname>Качанов</surname><given-names>Д. Ю.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><bold>Denis Yu. Kachanov</bold>, Dr. Med. Sci., Head of the Department of Clinical Oncology, Deputy Director of the Institute of Oncology, Radiology and Nuclear Medicine</p><p><italic>117997, Moscow, Samory Mashela St., 1</italic></p></bio><bio xml:lang="ru"><p><bold>Качанов Денис Юрьевич</bold>, д-р мед. наук, заведующий отделением клинической онкологии, заместитель директора Института онкологии, радиологии и ядерной медицины</p><p><italic>117997, Москва, ул. Саморы Машела, 1</italic></p></bio><email>Denis.Kachanov@fccho-moscow.ru</email><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation</institution></aff><aff><institution xml:lang="ru">ФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">B.V. Petrovsky National Scientific Center of Surgery</institution></aff><aff><institution xml:lang="ru">ФГБНУ «Российский научный центр хирургии им. акад. Б.В. Петровского»</institution></aff></aff-alternatives><aff-alternatives id="aff3"><aff><institution xml:lang="en">Moscow City Oncology Hospital No. 62 of the Department of Health of Moscow</institution></aff><aff><institution xml:lang="ru">Московская онкологическая больница №62 Департамента здравоохранения г. Москвы</institution></aff></aff-alternatives><aff-alternatives id="aff4"><aff><institution xml:lang="en">Russian Children’s Clinical Hospital of the N.I. Pigorov Russian National Research Medical University of Ministry of Healthcare of the Russian Federation</institution></aff><aff><institution xml:lang="ru">Российская детская клиническая больница ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России</institution></aff></aff-alternatives><aff-alternatives id="aff5"><aff><institution xml:lang="en">N.N. Blokhin National Medical Research Center of Oncology of Ministry of Healthcare of the Russian Federation</institution></aff><aff><institution xml:lang="ru">ФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2022-12-26" publication-format="electronic"><day>26</day><month>12</month><year>2022</year></pub-date><volume>21</volume><issue>4</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>31</fpage><lpage>44</lpage><history><date date-type="received" iso-8601-date="2022-04-29"><day>29</day><month>04</month><year>2022</year></date><date date-type="accepted" iso-8601-date="2022-05-11"><day>11</day><month>05</month><year>2022</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2022, «D. Rogachev NMRCPHOI»</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2022, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</copyright-statement><copyright-year>2022</copyright-year><copyright-holder xml:lang="en">«D. Rogachev NMRCPHOI»</copyright-holder><copyright-holder xml:lang="ru">ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://hemoncim.com/jour/article/view/606">https://hemoncim.com/jour/article/view/606</self-uri><abstract xml:lang="en"><p>Risk-adapted therapy is the standard of care for hepatoblastoma (HB). The aim of this study was to analyze the effectiveness of cisplatin monotherapy in patients with standard-risk HB. The study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. For the period 02.2012–12.2019 (95 months) 60 patients with standard-risk HB aged 0–8 years were treated within the framework of the cooperation of Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation and B.V. Petrovsky Russian Scientific Center of Surgery. The SIOPEL criteria were used for stratification into risk groups. Throughout the study period, standard-risk patients received therapy per SIOPEL-3 SR protocol, including cisplatin monotherapy. Survival was assessed by the Kaplan–Meier method. For the purposes of this study, overall survival (OS), event-free survival (EFS), where any modification of chemotherapy regimen towards its escalation were considered as an additional event, and progression-free survival (PFS) were calculated. The survival analysis was carried out on 15.01.2021; 54/60 (90%) patients were treated with cisplatin monotherapy and included in the final analysis. Median age at diagnosis was 11.3 (range 0.0–87.7) months. Male:female ratio – 0.86:1. Distribution by PRETEXT stages: I – 14 (25.9%), II – 30 (55.6%), III – 10 (18.5%) patients. The median alphafetoprotein level at the time of diagnosis was 162 979 (range 129–2 000 000) ng/ml. Modification of therapy without confirmed relapse/progression was required in 3/54 patients. Median follow-up was 47.1 (range 2–99) months. Among 54 patients 52 (96.3%) are alive, 2 (3.7%) patients died (1/2 – complications of surgical treatment). Relapses/progressions were noted in 4/54 (7.4%) patients, one of whom died due to disease progression. The three-year OS was 98.1% (95% confidence interval (CI) 94.6–100), EFS – 85.1% (95% CI 75.5–94.6), PFS – 90.5% (95% CI 82.5–98.4). Our data are consistent with the original studies of the SIOPEL group and convincingly confirm the effectiveness of cisplatin monotherapy in patients with HB of the standard risk group in the Russian Federation. Currently this regimen is incorporated into the national clinical guidelines of HB therapy and considered as a standard of care.</p></abstract><trans-abstract xml:lang="ru"><p>Риск-адаптированная терапия является стандартом лечения гепатобластомы (ГБ). Целью настоящего исследования явилось изучение эффективности монотерапии цисплатином у пациентов с ГБ группы стандартного риска. Данное исследование одобрено независимым этическим комитетом и утверждено решением ученого совета НМИЦ ДГОИ им. Дмитрия Рогачева. За период 02.2012–12.2019 (95 мес) в условиях ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России и в рамках программы межинститутского сотрудничества с ФГБНУ «РНЦХ им. акад. Б.В. Петровского» обследовались и получали лечение 60 пациентов с ГБ группы стандартного риска в возрасте от 0 до 8 лет. Для стратификации на группы риска использовались критерии группы SIOPEL. На протяжении всего периода исследования пациенты группы стандартного риска получали программную терапию по протоколу SIOPEL-3 SR, включающую цисплатин в монорежиме. Выживаемость оценивалась по методу Каплана–Майера. Анализировались общая выживаемость (ОВ), бессобытийная выживаемость (БСВ), где в качестве дополнительного события рассматривалось любое изменение в режиме химиотерапии в сторону его эскалации, и выживаемость без прогрессирования (ВБП). Оценка выживаемости проведена по состоянию на 15.01.2021. В анализ включены 54/60 (90%) пациента, получавших монотерапию цисплатином. Медиана возраста на момент постановки диагноза ГБ составила 11,3 (разброс 0,0–87,7) месяца. Соотношение мальчики:девочки составило 0,86:1. Распределение по стадиям PRETEXT: I – 14 (25,9%), II – 30 (55,6%), III – 10 (18,5%) пациентов. Медиана уровня альфа-фетопротеина на момент постановки диагноза составила 162 979 (разброс: 129–2 000 000) нг/мл. Модификация терапии, не связанная с рецидивом/прогрессией, потребовалась 3/54 пациентам. Медиана наблюдения составила 47,1 (разброс 2–99) мес. Из 54 пациентов, включенных в исследование, 52 (96,3%) живы, 2 (3,7%) погибли (1/2 – от осложнений хирургического лечения). Рецидивы/прогрессии отмечены у 4/54 (7,4%) пациентов, 1 из них погиб на фоне прогрессирования опухолевого процесса. Трехлетняя ОВ составила 98,1% (95% доверительный интервал (ДИ) 94,6–100), БСВ – 85,1% (95% ДИ 75,5–94,6), ВБП – 90,5% (95% ДИ 82,5–98,4). Полученные нами данные согласуются с оригинальными исследованиями группы SIOPEL и убедительно подтверждают эффективность монотерапии цисплатином у пациентов с ГБ группы стандартного риска в Российской Федерации. В настоящее время данная схема лечения включена в клинические рекомендации по лечению ГБ и может рассматриваться в качестве стандарта терапии.</p></trans-abstract><kwd-group xml:lang="en"><kwd>children</kwd><kwd>hepatoblastoma</kwd><kwd>alpha-fetoprotein</kwd><kwd>chemotherapy</kwd><kwd>cisplatin</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>гепатобластома</kwd><kwd>дети</kwd><kwd>альфа-фетопротеин</kwd><kwd>химиотерапия</kwd><kwd>цисплатин</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">1. Lim I.I.P., Bondoc A.J., Geller J.I., Tiao G.M. Hepatoblastoma – The Evolution of Biology, Surgery, and Transplantation. Children (Basel) 2018; 6 (1): 1. DOI:10.3390/children6010001</mixed-citation><mixed-citation xml:lang="ru">Lim I.I.P., Bondoc A.J., Geller J.I., Tiao G.M. Hepatoblastoma – The Evolution of Biology, Surgery, and Transplantation. Children (Basel) 2018; 6 (1): 1. 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