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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">10.21682/2311-1267-2019-6-3-44-53</article-id><article-id custom-type="elpub" pub-id-type="custom">nodgo-525</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>ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL STUDIES</subject></subj-group></article-categories><title-group><article-title>Меланотическая нейроэктодермальная опухоль младенцев: описание опыта НМИЦ ДГОИ им. Дмитрия Рогачева и обзор литературы</article-title><trans-title-group xml:lang="en"><trans-title>Melanotic neuroectodermal tumor of infancy: a description of the experience of Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology and literature review</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5489-1879</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сулейманова</surname><given-names>А. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Suleymanova</surname><given-names>A. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Амина Магомедовна Сулейманова</p><p>врач-детский онколог отделения хирургии № 2 (опухолей торакоабдоминальной локализации)</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Pediatric Oncologist Department of Surgery No. 2 (Tumors of Thoracoabdominal Localization) Research Institute of Pediatric Oncology and Hematology</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">aminasuleymanova313@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Озеров</surname><given-names>С. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Ozerov</surname><given-names>S. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-нейрохирург отделения онкологии и детской хирургии, главный научный сотрудник отдела нейроонкологии</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Neurosurgeon of the Department of Oncology and Pediatric Surgery, Chief Researcher of the Department of Neurooncology</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">Gagara3@yandex.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/0000-0002-9375-7517</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Рощин</surname><given-names>В. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Roshchin</surname><given-names>V. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-патологоанатом патологоанатомического отделения</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Pathologist of the Pathology Department</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">vit1982@list.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/0000-0002-4451-3233</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Грачёв</surname><given-names>Н. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Grachev</surname><given-names>N. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., врач-онколог, заведующий отделением онкологии и детской хирургии, руководитель группы хирургии головы и шеи и реконструктивно-пластической хирургии отдела хирургии и анестезиологии, заместитель директора Института онкологии, радиологии и ядерной медицины</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Oncologist, Head of the Department of Oncology and Pediatric Surgery, Head of the Head and Neck Surgery and Reconstructive Plastic Surgery Unit of the Department of Surgery and Anesthesiology, Deputy Director of the Institute of Oncology, Radiology and Nuclear Medicine</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">nick-grachev@yandex.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/0000-0001-6313-6712</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Большаков</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Bolshakov</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-ортопед-травматолог отделения онкологии и детской хирургии</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Orthopedic and Traumatologist of the Department of Oncology and Pediatric Surgery</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">bolshakovn@yandex.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/0000-0003-1308-8622</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Друй</surname><given-names>А. Е.</given-names></name><name name-style="western" xml:lang="en"><surname>Druy</surname><given-names>A. E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач клинической лабораторной диагностики лаборатории цитогенетики и молекулярной генетики</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Doctor of Clinical Laboratory Diagnostics Laboratory of Cytogenetics and Molecular Genetics</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">dr-drui@yandex.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/0000-0001-7317-7104</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Терещенко</surname><given-names>Г. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Tereshchenko</surname><given-names>G. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., врач-рентгенолог, заведующая рентгенологическим отделением</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Radiologist, Head of the Radiology Department</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">galina.tereshenko@fccho-moscow.ru</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Коновалов</surname><given-names>Д. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Konovalov</surname><given-names>D. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., заведующий патологоанатомическим отделением</p><p>117997, Москва, ул. Саморы Машела, 1</p><p> </p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Pathologist, Head of the Pathology Department</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">pathmorf@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/0000-0002-7846-3473</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сагоян</surname><given-names>Г. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Sagoyan</surname><given-names>G. B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>врач-детский онколог отделения хирургии № 2 (опухолей торакоабдоминальной локализации) НИИ детской онкологии и гематологии</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Pediatric Oncologist Department of Surgery No. 2 (Tumors of Thoracoabdominal Localization)</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">sagoyan-garik@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/0000-0002-3767-4477</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Шаманская</surname><given-names>Т. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Shamanskaya</surname><given-names>T. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., ученый секретарь Института онкологии, радиологии и ядерной медицины</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Cand. of Sci. (Med.), Academic Secretary of the Institute of Oncology, Radiology and Nuclear Medicine</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">shamanskaya.tatyana@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3704-8783</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Качанов</surname><given-names>Д. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Kachanov</surname><given-names>D. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., заведующий отделением клинической онкологии, заместитель директора Института онкологии, радиологиии ядерной медицины, SPIN-код: 9878-5540</p><p>117997, Москва, ул. Саморы Машела, 1</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Head of the Department of Clinical Oncology and Department of Embryonic Tumor Research &amp; Deputy Director of the Institute of Oncology, Radiology and Nuclear Medicine, SPIN-code: 9878-5540</p><p>1 Samory Mashela St., Moscow, 117997</p></bio><email xlink:type="simple">Denis.Kachanov@fccho-moscow.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/0000-0001-6131-1783</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, заместитель директора по научной и лечебной работе – директор НИИ детской онкологии и гематологии аппарата управления, исполнительный директор РОО НОДГО</p><p>115478, Москва, Каширское шоссе, 23</p></bio><bio xml:lang="en"><p>Dr. of Sci. (Med.), Professor, Deputy Director for Research and Clinical Work – Director</p><p>23 Kashirskoe Shosse, Moscow, 115478</p></bio><email xlink:type="simple">clinoncology@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>НИИ детской онкологии и гематологии ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Research Institute of Pediatric Oncology and Hematology of 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-2"><aff xml:lang="ru"><institution>ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2019</year></pub-date><pub-date pub-type="epub"><day>22</day><month>09</month><year>2019</year></pub-date><volume>6</volume><issue>3</issue><fpage>44</fpage><lpage>53</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Сулейманова А.М., Озеров С.С., Рощин В.Ю., Грачёв Н.С., Большаков Н.А., Друй А.Е., Терещенко Г.В., Коновалов Д.М., Сагоян Г.Б., Шаманская Т.В., Качанов Д.Ю., Варфоломеева С.Р., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Сулейманова А.М., Озеров С.С., Рощин В.Ю., Грачёв Н.С., Большаков Н.А., Друй А.Е., Терещенко Г.В., Коновалов Д.М., Сагоян Г.Б., Шаманская Т.В., Качанов Д.Ю., Варфоломеева С.Р.</copyright-holder><copyright-holder xml:lang="en">Suleymanova A.M., Ozerov S.S., Roshchin V.Y., Grachev N.S., Bolshakov N.A., Druy A.E., Tereshchenko G.V., Konovalov D.M., Sagoyan G.B., Shamanskaya T.V., Kachanov D.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/525">https://journal.nodgo.org/jour/article/view/525</self-uri><abstract><p>Меланотическая нейроэктодермальная опухоль младенцев (МНЭО) относится к редким опухолям с неопределенным биологическим поведением. Наиболее часто МНЭО локализуются в области головы и шеи. Стандартом терапии является радикальное хирургическое удаление опухоли, что зачастую сопряжено с высокими рисками тяжелых послеоперационных осложнений за счет локализации опухоли. В случае метастатической, местно-распространенной формы заболевания или в случае развития рецидивов используются альтернативные методы лечения, включающие химиотерапию, лучевую терапию. Целью данной публикации является изучение клинических и морфологических характеристик опухоли, оценка эффективности различных вариантов лечения, а также описание 2 клинических случаев МНЭО у детей раннего возраста.</p></abstract><trans-abstract xml:lang="en"><p>Melanotic neuroectodermal tumor of infancy (MNTI) is a rare type of tumor with uncertain clinical behavior. The tumor commonly occurred in the head and neck region. Radical surgical resection is the standard of treatment, which is often associated with high risks of severe postoperative complications due to tumor localization. Alternative methods of treatment including chemotherapy/radiotherapy are used in metastatic, locally advanced and relapsed cases of MNTI. The aim of this study was to analyze clinical and morphological characteristics of the tumor, evaluate the effectiveness of various treatment options, and to describe two clinical cases of MNTI.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>меланотическая нейроэктодермальная опухоль младенцев</kwd><kwd>редкие опухоли</kwd><kwd>дети</kwd><kwd>меланотическая прогонома</kwd><kwd>доброкачественные опухоли</kwd></kwd-group><kwd-group xml:lang="en"><kwd>melanotic neuroectodermal tumor of infancy</kwd><kwd>rare tumor</kwd><kwd>children</kwd><kwd>melanotic progonoma</kwd><kwd>benign tumor</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">Barnes L., Eveson J.W., Reichart P., Sidransky D. World Health Organization classification of tumors. Pathology and genetics, malignant soft tissue tumors, vol. 149. 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