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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">227</article-id><article-id pub-id-type="doi">10.24287/1726-1708-2019-18-1-62-72</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">Optic pathway gliomas in children: prognostic factors, response assessment, role of carboplatin and vincristine chemotherapy regime</article-title><trans-title-group xml:lang="ru"><trans-title>Глиомы зрительного пути у детей: прогностические факторы, оценка ответа и роль двухкомпонентной химиотерапии</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2977-665X</contrib-id><name-alternatives><name xml:lang="en"><surname>Valiakhmetova</surname><given-names>E. F.</given-names></name><name xml:lang="ru"><surname>Валиахметова</surname><given-names>Э. Ф.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Endge F. Valiakhmetova - MD, pediatric oncologist.</p><p>125047, Moscow, 4th Tverskaya-Yamskaya st., 16</p></bio><bio xml:lang="ru"><p>Валиахметова Эндже Фаварисовна - врач - детский онколог.</p><p>125047, Москва, 4-я Тверская-Ямская ул., 16</p></bio><email>andgeval@gmail.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3232-2322</contrib-id><name-alternatives><name xml:lang="en"><surname>Budanov</surname><given-names>O. I.</given-names></name><name xml:lang="ru"><surname>Быданов</surname><given-names>О. И.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Gorelyshev</surname><given-names>S. K.</given-names></name><name xml:lang="ru"><surname>Горелышев</surname><given-names>С. К.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff3"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Serova</surname><given-names>N. K.</given-names></name><name xml:lang="ru"><surname>Серова</surname><given-names>Н. К.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Lasareva</surname><given-names>L. A.</given-names></name><name xml:lang="ru"><surname>Лазарева</surname><given-names>Л. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Shiskina</surname><given-names>L. V.</given-names></name><name xml:lang="ru"><surname>Шишкина</surname><given-names>Л. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Mazerkina</surname><given-names>N. A.</given-names></name><name xml:lang="ru"><surname>Мазеркина</surname><given-names>Н. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Trunin</surname><given-names>Y. Y.</given-names></name><name xml:lang="ru"><surname>Трунин</surname><given-names>Ю. Ю.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-4412-5019</contrib-id><name-alternatives><name xml:lang="en"><surname>Chulkova</surname><given-names>S. V.</given-names></name><name xml:lang="ru"><surname>Чулкова</surname><given-names>С. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff4"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Grishchenko</surname><given-names>N. V.</given-names></name><name xml:lang="ru"><surname>Грищенко</surname><given-names>Н. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff4"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3904-8530</contrib-id><name-alternatives><name xml:lang="en"><surname>Egorova</surname><given-names>A. V.</given-names></name><name xml:lang="ru"><surname>Егорова</surname><given-names>А. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff4"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7750-5216</contrib-id><name-alternatives><name xml:lang="en"><surname>Papusha</surname><given-names>L. I.</given-names></name><name xml:lang="ru"><surname>Папуша</surname><given-names>Л. И.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2322-5734</contrib-id><name-alternatives><name xml:lang="en"><surname>Novichkova</surname><given-names>G. А.</given-names></name><name xml:lang="ru"><surname>Новичкова</surname><given-names>Г. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Karachunskii</surname><given-names>A. I.</given-names></name><name xml:lang="ru"><surname>Карачунский</surname><given-names>А. И.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Moscow</p></bio><bio xml:lang="ru"><p>Москва</p></bio><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff2"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">N.N. Burdenko National Medical Research Center of neurosurgery Ministry of Healthcare of Russian Federation</institution></aff><aff><institution xml:lang="ru">ФГАУ «Национальный медицинский исследовательский центр нейрохирургии им. академика Н.Н. Бурденко» Минздрава России</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">Dmitriy Rogachev National Medical Research Center of Pediatric Hematology, Oncology, Immunology Ministry of Healthcare of 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="aff3"><aff><institution xml:lang="en">N.I. Pirogov Russian National Research Medical University</institution></aff><aff><institution xml:lang="ru">ФГБОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России</institution></aff></aff-alternatives><aff-alternatives id="aff4"><aff><institution xml:lang="en">N.N. Blokhin National Medical Research Center of oncology Ministry of Healthcare of 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="2019-04-21" publication-format="electronic"><day>21</day><month>04</month><year>2019</year></pub-date><volume>18</volume><issue>1</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>62</fpage><lpage>72</lpage><history><date date-type="received" iso-8601-date="2019-04-21"><day>21</day><month>04</month><year>2019</year></date><date date-type="accepted" iso-8601-date="2019-04-21"><day>21</day><month>04</month><year>2019</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2019, «D. Rogachev NMRCPHOI»</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2019, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</copyright-statement><copyright-year>2019</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/227">https://hemoncim.com/jour/article/view/227</self-uri><abstract xml:lang="en"><p>Low grade gliomas (LGG) constitute 35-40% of all primary central nervous system (CNS) tumors in children, at least 10-12% of them affecting the optic pathway. The complexity of the localization and predominantly the diffuse type of growth of these tumors make the neurosurgical treatment not appropriate in most cases, which requires conservative treatment.</p><p><bold>Objective: </bold>to analyze the results of carboplatin and vincristine chemotherapy (CT) regime in children with optic pathway gliomas (OPG). The study was approved by the Independent Ethics Committee of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. In the study was included patients registered at the Burdenko Neurosurgery Institute from January 1, 2003 till December 31, 2015 with optic pathway glioma from 0 to 18 years old that were treated with combined carboplatin and vincristine chemotherapy. All patients were divided into 2 groups: the first group included patients who had newly diagnosed OPG (group 1), the second group included patients with OPG with radiological progression following observation times (group 2). Evaluation of the response to treatment was carried out on the 24th week and after completion of treatment by delineation of each slice of the MRI images in OsiriX MD software (© Pixmeo Sari, Switzerland) with subsequent automatic volume calculation.</p><p><bold>Results:</bold> 104 patients were included in the analysis. 69 children were included to group 1, 35 patients in group 2. 60% of patients were boys, about a third of children were less than 18 months old, 17% of patients had Neurofibromatosis type I, 24% of patients had diencephalic cachexia. A biopsy of the tumor was performed in 76% of the patients, 54% of the patients had a piloid astrocytoma and 23% had a pilomixoid astrocytoma. The progression after the start of the chemotherapy was in 30 (28.9%) patients. 5-year event free survival (EFS) and overall survival (OS) were 58 ± 6% and 97 ± 2%, respectively. 5-year EFS was not statistically significantly different in patients of groups 1 and 2. According to our data, the age, histology of the tumor, the response to the 24th week of CT are independent prognostic factors.</p></abstract><trans-abstract xml:lang="ru"><p>Глиальные опухоли (глиомы) низкой степени злокачественности составляют 35-40% всех первичных опухолей центральной нервной системы у детей; не менее 10-12% из них поражают зрительный путь. Из-за сложности локализации и преимущественно диффузного типа роста этих опухолей в большинстве случаев, кроме нейрохирургического, требуется консервативное лечение.</p><p><bold>Цель исследования</bold>: оценить эффективность химиотерапии препаратами карбоплатин и винкристин у детей с глиомами зрительного пути. Данное исследование поддержано Независимым этическим комитетом и утверждено решением Ученого совета НМИЦ ДГОИ. В исследование были включены больные в возрасте от 0 до 18 лет, зарегистрированные в НМИЦ нейрохирургии им. академика Н.Н. Бурденко в период с 01.01.2003 по 31.12. 2015 с первичным диагнозом «глиома зрительного пути», которым проводили противоопухолевую химиотерапию (ПХТ) карбоплатином и винкристином. Этих больных разделили на две группы: в 1-ю группу вошли пациенты, которым ПХТ назначили на момент первичной диагностики; пациентам 2-й группы - при обнаружении продолженного роста опухоли. Оценку ответа на лечение проводили на 24-й нед. и после завершения лечения путем оконтуривания каждого среза МР-томограммы в программном обеспечении OsiriX MD (© Pixmeo Sarl, Швейцария) с последующим автоматическим подсчетом объема. Всего были зарегистрированы и включены в анализ 104 пациента (60% из них - мальчики); около трети детей - в возрасте менее 18 мес.: в 1-й группе - 69 детей; во 2-й - 35. У 17% больных был обнаружен нейрофиброматоз I типа; 24% пациентов имели диэнцефальную кахексию на момент регистрации в исследовании. У 76% больных провели биопсию опухоли: у 54% из них диагностировали пилоидную астроцитому; у 23% - пиломиксоидную астроцитому. Продолженный рост после окончания ПХТ был зафиксирован у 30 (28,9%) пациентов. Для всех больных 5-летняя бессобытийная и общая выживаемость составили 58 ± 6% и 97 ± 2% соответственно. У пациентов 1-й и 2-й групп 5-летняя бессобытийная выживаемость статистически значимо не отличалась. По нашим данным, возраст, гистологический вариант, ответ на 24-й нед. ПХТ - независимые прогностические факторы.</p></trans-abstract><kwd-group xml:lang="en"><kwd>optic pathway gliomas</kwd><kwd>children</kwd><kwd>chemotherapy</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>глиомы зрительного пути</kwd><kwd>дети</kwd><kwd>полихимиотерапия</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.	Jahraus C.D., Tarbell N.J. Optic pathway gliomas. 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