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<article article-type="research-article" dtd-version="1.3" 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" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">nodgo</journal-id><journal-title-group><journal-title xml:lang="ru">Российский журнал детской гематологии и онкологии (РЖДГиО)</journal-title><trans-title-group xml:lang="en"><trans-title>Russian Journal of Pediatric Hematology and Oncology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2311-1267</issn><issn pub-type="epub">2413-5496</issn><publisher><publisher-name>LTD “Graphica”</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21682/2311-1267-2022-9-2-93-105</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-834</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>CLINICAL CASES</subject></subj-group></article-categories><title-group><article-title>Экстраокулярная ретинобластома: обзор литературы и клинический случай (интракраниальная ретинобластома с вовлечением зрительных трактов)</article-title><trans-title-group xml:lang="en"><trans-title>Extraocular retinoblastoma: literature review and clinical case (intracranial retinoblastoma involving the visual tracts)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3263-6655</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ушакова</surname><given-names>Т. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Ushakova</surname><given-names>T. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Татьяна Леонидовна Ушакова, д.м.н., ведущий научный сотрудник детского онкологического отделения хирургических методов лечения с проведением химиотерапии № 1 (опухолей головы и шеи) НИИ детской онкологии и гематологии</p><p>SPIN-код: 2065-8779</p><p>115478, Москва, Каширское шоссе, 23</p><p>125993, Москва, ул. Баррикадная, 2/1, стр. 1</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Leading Researcher Pediatric Oncology Department of Surgical Treatment with Chemotherapy No. 1 (Head and Neck Tumors) of the Research Institute of Pediatric Oncology and Hematology; Professor at the Department of Pediatric Oncology named after Academician L.A. Durnov</p><p>SPIN-code: 2065-8779</p><p>23 Kashirskoe Shosse, Moscow, 115478</p><p>Bld. 1, 2/1 Barrikadnaya St., Moscow, 125993</p></bio><email xlink:type="simple">ushtat07@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8096-0874</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Поляков</surname><given-names>В. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Polyakov</surname><given-names>V. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>академик РАН, д.м.н., профессор, советник директора и заведующий детским онкологическим отделением хирургических методов лечения с проведением химиотерапии № 1 (опухолей головы и шеи) НИИ детской онкологии и гематологии; профессор кафедры оториноларингологии педиатрического факультета</p><p>SPIN-код: 8606-3120</p><p>115478, Москва, Каширское шоссе, 23</p><p>125993, Москва, ул. Баррикадная, 2/1, стр. 1</p><p>117997, Москва, ул. Островитянова, 1</p></bio><bio xml:lang="en"><p>Academician of RAS, Dr. of Sci. (Med.), Professor, Advisor to the Director and Head of the Pediatric Oncology Department of Surgical Treatment Methods with Chemotherapy No. 1 (Head and Neck Tumors) of the Research Institute of Pediatric Oncology and Hematology; Head of the Pediatric Oncology Department named after Academician L.A. Durnov; Professor of the Department of Otorhinolaryngology Faculty of Pediatrics</p><p>SPIN-code: 8606-3120</p><p>23 Kashirskoe Shosse, Moscow, 115478</p><p>Bld. 1, 2/1 Barrikadnaya St., Moscow, 125993</p><p>1 Ostrovityanova St., Moscow, 117997</p></bio><email xlink:type="simple">vgp-04@mail.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-8356-0625</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Родина</surname><given-names>А. Д.</given-names></name><name name-style="western" xml:lang="en"><surname>Rodina</surname><given-names>A. D.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог детского онкологического отделения хирургических методов лечения с проведением химиотерапии № 1 (опухолей головы и шеи)</p><p>SPIN-код: 5915-0784</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Pediatric Oncologist Pediatric Oncology Department of Surgical Treatment with Chemotherapy No. 1 (Head and Neck Tumors) of the Research Institute of Pediatric Oncology and Hematology</p><p>SPIN-code: 5915-0</p><p>Russia; 23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">dr.rodinaAD@yandex.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6001-4963</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Власова</surname><given-names>В. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Vlasova</surname><given-names>V. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>студент международного факультета</p><p>117997, Москва, ул. Островитянова, 1</p></bio><bio xml:lang="en"><p>Student of the International Faculty</p><p>Ostrovityanova St., Moscow, 117997</p></bio><email xlink:type="simple">vlasova12345678@icloud.com</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1778-9867</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Бланкова</surname><given-names>Т. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Blankova</surname><given-names>T. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>студент лечебного факультета</p><p>117997, Москва, ул. Островитянова, 1</p></bio><bio xml:lang="en"><p>Student of the Faculty of Medicine</p><p>Ostrovityanova St., Moscow, 117997</p></bio><email xlink:type="simple">blankova1998@gmail.com</email><xref ref-type="aff" rid="aff-4"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8827-748X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Артеменко</surname><given-names>Ю. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Artemenko</surname><given-names>Yu. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-рентгенолог, врач ультразвуковой диагностики рентгенодиагностического отделения отдела лучевых методов диагностики и терапии опухолей НИИ детской онкологии и гематологии</p><p>SPIN-код: 5956-7279</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Radiologist, Ultrasound Specialist of the Department of Radiology of the Department of Radiation Methods for the Diagnosis and Treatment of Tumors of the Research Institute of Pediatric Oncology and Hematology</p><p>SPIN-code: 5956-7279</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">jul.artko@gmail.com</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-7630-7496</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Михайлова</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Mikhailova</surname><given-names>E. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующая рентгенодиагностическим отделением отдела лучевых методов диагностики и лечения опухолей НИИ детской онкологии и гематологии</p><p>SPIN-код: 2880-1263</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Head of the Department of Radiology of the Department of Radiation Methods for the Diagnosis and Treatment of Tumors of the Research Institute of Pediatric Oncology and Hematology</p><p>SPIN-code: 2880-1263</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">elena_1357@mail.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8476-7879</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Крылов</surname><given-names>А. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Krylov</surname><given-names>A. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующий лабораторией радиоизотопной диагностики отдела радиоизотопной диагностики и терапии НИИ клинической и экспериментальной радиологии</p><p>SPIN-код: 4254-3930</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p> Cand. of Sci. (Med.), Head of the Laboratory of Radioisotope Diagnostics Department of Radioisotope Diagnostics and Therapy of the Research Institute of Clinical and Experimental Radiology</p><p>SPIN-code: 4254-3930</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">krilovas@rambler.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3576-6156</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Оджарова</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Odzharova</surname><given-names>A. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., старший научный сотрудник отделения позитронной эмиссионной томографии НИИ клинической и экспериментальной радиологии</p><p>SPIN-код: 1074-3862</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Senior Researcher Positron Emission Tomography Department of the Research Institute of Clinical and Experimental Radiology</p><p>SPIN-code: 1074-3862</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">odzharova07@yandex.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-2391-4142</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Григоренко</surname><given-names>В. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Grigorenko</surname><given-names>V. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-радиотерапевт, заведующий радиологическим отделением НИИ детской онкологии и гематологии</p><p>SPIN-код: 4263-1844</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p> Radiotherapist, Head of the Radiological Department of the Research Institute of Pediatric Oncology and Hematology</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">oncogrigorenko@yandex.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5663-4032</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шириков</surname><given-names>Е. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Shirikov</surname><given-names>E. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-радиотерапевт радиологического отделения НИИ детской онкологии и гематологии</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Radiotherapist Radiological Department of the Research Institute of Pediatric Oncology and Hematology</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">Evgeniyshirikov88@gmail.com</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3852-3969</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Козлов</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Kozlov</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-патологоанатом патологоанатомического отделения отдела морфологической и молекулярно-генетической диагностики опухолей</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>N.A. Kozlov: Cand. of Sci. (Med.), Pathologist Pathological Department of the Department of Morphological and Molecular-Genetic Diagnostics of Tumors</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">newbox13@mail.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0194-7645</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Гаспарян</surname><given-names>Т. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Gasparyan</surname><given-names>T. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-нейрохирург отделения нейрохирургического – онкологического НИИ клинической онкологии; научный сотрудник нейрохирургического отделения</p><p>SPIN-код: 5198-5167</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Neurosurgeon Neurosurgical Department – Oncological of the Research Institute of Clinical Oncology</p><p>SPIN-code: 5198-5167</p><p>23 Kashirskoe Shosse, Moscow, 115478</p><p>80 Volokolamskoe Shosse, Moscow,125367</p></bio><email xlink:type="simple">drkoko@yandex.ru</email><xref ref-type="aff" rid="aff-5"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Бекяшев</surname><given-names>А. Х.</given-names></name><name name-style="western" xml:lang="en"><surname>Bekyashev</surname><given-names>A. Kh.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., врач-нейрохирург, заведующий отделением нейрохирургическим – онкологическим НИИ клинической онкологии</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Doct. of Sci. (Med.), Neurosurgeon, Head of the Neurosurgical Department – Oncological of the Research Institute of Clinical Oncology</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">abekyashev@gmail.com</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-5706-9496</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Докучаева</surname><given-names>Я. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Dokuchaeva</surname><given-names>Ya. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-ординатор первого года по специальности «детская онкология» НИИ детской онкологии и гематологии</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>first-year Resident Doctor in the specialty “Pediatric Oncology” of the Research Institute of Pediatric Oncology and Hematology</p><p>Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">ljrexftdfzyf98@mail.ru</email><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0001-6131-1783</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Варфоломеева</surname><given-names>С. Р.</given-names></name><name name-style="western" xml:lang="en"><surname>Varfolomeeva</surname><given-names>S. R.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., профессор, директор НИИ детской онкологии и гематологии; профессор кафедры детской онкологии им. акад. Л.А. Дурнова</p><p>115478, Москва, Каширское шоссе, 23</p><p>125993, Москва, ул. Баррикадная, 2/1, стр. 1</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Director of the Research Institute of Pediatric Oncology and Hematology;  Professor at the Department of Pediatric Oncology named after Academician L.A. Durnov</p><p>23 Kashirskoe Shosse, Moscow, 115478</p><p>Bld. 1, 2/1 Barrikadnaya St., Moscow, 125993</p></bio><email xlink:type="simple">s.varfolomeeva@ronc.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России; ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia; Russian Medical Academy of Continuous Professional Education, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России; ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России; ФГАОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia; Russian Medical Academy of Continuous Professional Education, Ministry of Health of Russia; N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-4"><aff xml:lang="ru"><institution>ФГАОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-5"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России; ФГБНУ «Научный центр неврологии» Министерства образования и науки России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>N.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia; Scientific Center of Neurology, Ministry of Education and Science of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2022</year></pub-date><pub-date pub-type="epub"><day>16</day><month>08</month><year>2022</year></pub-date><volume>9</volume><issue>2</issue><fpage>93</fpage><lpage>105</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Ушакова Т.Л., Поляков В.Г., Родина А.Д., Власова В.Е., Бланкова Т.И., Артеменко Ю.В., Михайлова Е.В., Крылов А.С., Оджарова А.А., Григоренко В.А., Шириков Е.И., Козлов Н.А., Гаспарян Т.Г., Бекяшев А.Х., Докучаева Я.Ю., Варфоломеева С.Р., 2022</copyright-statement><copyright-year>2022</copyright-year><copyright-holder xml:lang="ru">Ушакова Т.Л., Поляков В.Г., Родина А.Д., Власова В.Е., Бланкова Т.И., Артеменко Ю.В., Михайлова Е.В., Крылов А.С., Оджарова А.А., Григоренко В.А., Шириков Е.И., Козлов Н.А., Гаспарян Т.Г., Бекяшев А.Х., Докучаева Я.Ю., Варфоломеева С.Р.</copyright-holder><copyright-holder xml:lang="en">Ushakova T.L., Polyakov V.G., Rodina A.D., Vlasova V.E., Blankova T.I., Artemenko Y.V., Mikhailova E.V., Krylov A.S., Odzharova A.A., Grigorenko V.A., Shirikov E.I., Kozlov N.A., Gasparyan T.G., Bekyashev A.K., Dokuchaeva Y.Y., Varfolomeeva S.R.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://journal.nodgo.org/jour/article/view/834">https://journal.nodgo.org/jour/article/view/834</self-uri><abstract><p>Актуальность. Ретинобластома (РБ) – наиболее распространенная первичная внутриглазная опухоль у детей. Встречаемость РБ составляет от 1:14 000 до 1:20 000 новорожденных. На сегодняшний день показатели выживаемости больных РБ достигают почти 100 % при своевременной и адекватной диагностике. Среди онкологических заболеваний детского возраста на долю РБ приходится 2,3–4,5 % и 85–90 % – среди внутриглазных опухолей у детей. По объему распространения опухоли выделяют интра- и экстраокулярную формы заболевания. Экстраокулярная РБ – это распространение опухоли за пределы глаза с инвазией тканей орбиты, а также возможное вовлечение смежных областей, в том числе интракраниальных без и с регионарными и/или дистанционными метастазами. Основным методом лечения детей с экстраокулярной РБ является неоадъювантная химиотерапия (ХТ) с планированием оперативного вмешательства и адъювантной терапии. Проведение высокодозной ХТ (ВДХТ) с аутотрансплантацией гемопоэтических стволовых клеток (ауто-ТГСК) позволяет увеличить безрецидивную выживаемость у пациентов без метастатического поражения головного и спинного мозга, но при их поражении прогноз выживаемости крайне неблагоприятный, характер заболевания носит рецидивирующий характер со 100 % летальностью.Описание клинического случая. Мы сообщаем о случае двусторонней РБ: OD – с экстраокулярным и интракраниальным распространением опухоли по зрительному нерву с поражением хиазмы и переходом на начальные отделы зрительных трактов. Вторичная глаукома. OS – с интраокулярным ростом опухоли у ребенка 3 лет. После неоадъювантной ХТ, в том числе интратекальной, была выполнена одномоментная двухэтапная операция в объеме костно-пластической (височно-орбито- зигоматической) птериональной краниотомии справа с прехиазмальной резекцией правого зрительного нерва и энуклеацией правого глаза. В послеоперационном периоде проведена адъювантная ХТ с последующей ВДХТ с ауто-ТГСК. Лучевая терапия стала завершающим этапом лечения. Последовательно проведены брахитерапия на опухоль OS и дистанционная лучевая терапия на краниоспинальную область, правую орбиту, культю зрительного нерва с хиазмой и ножку гипофиза. Через 14 мес от начала лечения и через 5 мес после его окончания был выявлен лептоменингеальный рецидив заболевания.Заключение. Редкие наблюдения РБ с поражением зрительных трактов не позволяют достаточно изучить особенности течения опухолевого процесса, а также разработать единый эффективный подход к противоопухолевому лечению. Среди причин смертности у больных с экстраокулярной РБ основной является метастазирование в головной и спинной мозг.</p></abstract><trans-abstract xml:lang="en"><p>Relevance. Retinoblastoma (RB) is the most common primary intraocular tumor in children. The incidence of RB ranges from 1:14,000 to 1:20,000 newborns. To date, the survival rates of patients with RB reach almost 100 % with timely and adequate diagnosis. Among childhood oncological diseases, RB accounts for 2.3–4.5 % and 85–90 % among intraocular tumors in children. According to the volume of tumor spread, intra- and extraocular forms of the disease are distinguished. Extraocular RB is the spread of the tumor beyond the eye with invasion of the orbital tissues, as well as the possible involvement of adjacent areas, including intracranial without and with regional and/or remote metastases. The main method of treatment of children with extraocular RB is neoadjuvant chemotherapy (CT) with planning of surgical intervention and adjuvant therapy. High-dose CT (HDCT) with autologous hematopoietic stem cell transplantation (auto-HSCT), it allows to increase relapse-free survival in patients without metastatic lesions of the brain and spinal cord, but with their defeat, the prognosis of survival is extremely unfavorable, the nature of the disease is recurrent with 100 % lethality.Description of the clinical case. We report a case of bilateral RB: OD – with extraocular and intracranial spread of the tumor along the optic nerve with a lesion of the chiasm and a transition to the initial parts of the visual tracts. Secondary glaucoma. OS – with intraocular tumor growth in a 3-year-old child. After neoadjuvant chemotherapy, including intrathecal, a simultaneous two-stage operation was performed in the volume of bone-plastic (temporo-orbito-zygomatic) pterionic craniotomy on the right with prechiasmal resection of the right optic nerve and enucleation of the right eye. In the postoperative period, adjuvant chemotherapy was performed, followed by HDCT with auto-HSCT. Radiation therapy has become the final stage of treatment. Brachytherapy for OS tumor and remote radiotherapy for craniospinal region, right orbit, optic nerve stump with chiasm and pituitary pedicle were successively performed. After 14 months from the beginning of treatment and 5 months after its completion, a leptomeningeal relapse of the disease was detected.Conclusion. Rare observations of RB with damage to the visual tracts do not allow us to sufficiently study the features of the course of the tumor process, as well as to develop a single effective approach to antitumor treatment. Among the causes of mortality in patients with extraocular RB, the main one is metastasis, metastasis in the brain and spinal cord.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>офтальмоонкология</kwd><kwd>детская онкология</kwd><kwd>экстраокулярная ретинобластома</kwd><kwd>зрительный тракт</kwd><kwd>клинический случай</kwd></kwd-group><kwd-group xml:lang="en"><kwd>ophthalmooncology</kwd><kwd>pediatric oncology</kwd><kwd>extraocular retinoblastoma</kwd><kwd>visual tract</kwd><kwd>clinical case</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Ishaq H., Patel B.C. Retinoblastoma. [Updated 2021 Nov 2]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK545276/.</mixed-citation><mixed-citation xml:lang="en">Ishaq H., Patel B.C. Retinoblastoma. [Updated 2021 Nov 2]. 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