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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">medsovet</journal-id><journal-title-group><journal-title xml:lang="ru">Медицинский Совет</journal-title><trans-title-group xml:lang="en"><trans-title>Meditsinskiy sovet = Medical Council</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2079-701X</issn><issn pub-type="epub">2658-5790</issn><publisher><publisher-name>REMEDIUM GROUP Ltd.</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21518/ms2024-070</article-id><article-id custom-type="elpub" pub-id-type="custom">medsovet-8303</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>ПРАКТИКА</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>PRACTICE</subject></subj-group></article-categories><title-group><article-title>Редкое онкологическое заболевание у ребенка с манифестацией в лор-органах</article-title><trans-title-group xml:lang="en"><trans-title>A rare oncological disease in a child with manifestation in the ENT organs</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1355-813X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Андрианов</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Andrianov</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Андрианов Алексей Владимирович, младший научный сотрудник отдела патологии верхних дыхательных путей</p><p>190013, Санкт-Петербург, ул. Бронницкая, д. 9</p></bio><bio xml:lang="en"><p>Aleksei V. Andrianov, Junior Researcher at the Department of Pathology of the Upper Respiratory Tract,</p><p>9, Bronnitskaya St., St Petersburg, 190013, Russia</p></bio><email xlink:type="simple">andrianov.w@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/0000-0001-8883-498X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Дроздова</surname><given-names>М. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Drozdova</surname><given-names>M. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Дроздова Марина Владимировна, д.м.н., ведущий научный сотрудник отдела патологии верхних дыхательных путей, заведующая отделением реконструктивной хирургии </p><p>190013, Санкт-Петербург, ул. Бронницкая, д. 9</p></bio><bio xml:lang="en"><p>Marina V. Drozdova, Dr. Sci. (Med.), Senior Researcher at the Department of Pathology of the Upper Respiratory Tract, Head of the Department of Reconstructive Surgery</p><p>9, Bronnitskaya St., St Petersburg, 190013, Russia</p></bio><email xlink:type="simple">drozdova1504@yandex.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/0000-0002-3377-8711</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Алексеенко</surname><given-names>С. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Alekseenko</surname><given-names>S. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Алексеенко Светлана Иосифовна, д.м.н., старший научный сотрудник, Санкт-Петербургский научно-исследовательский институт уха, горла, носа и речи; заведующая лор-отделением, Детский городской многопрофильный клинический центр высоких медицинских технологий имени К.А. Раухфуса; доцент кафедры оториноларингологии, Северо-Западный государственный медицинский университет имени И.И. Мечникова;</p><p>190013, Санкт-Петербург, ул. Бронницкая, д. 9; 193036,  Санкт-Петербург, Лиговский проспект, д. 8; 191015, Санкт-Петербург, ул. Кирочная, д. 41</p></bio><bio xml:lang="en"><p>Svetlana I. Alekseenko, Dr. Sci. (Med.), Senior Researcher, Saint Petersburg Research Institute of Ear, Throat, Nose and Speech; Head of the ENT Department, Children’s City Multidisciplinary Clinical Center of High Medical Technologies named after K.A. Rauhfus; Associate Professor of the Department of Otorhinolaryngology, NorthWestern State Medical University named after I.I. Mechnikov;</p><p>9, Bronnitskaya St., St Petersburg, 190013, Russia; 8, Ligovsky Ave., St Petersburg, 193036, Russia; 41, Kirochnaya St., St Petersburg, 191015, Russia</p></bio><email xlink:type="simple">svolga-lor@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/0009-0000-1168-1786</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Васильева</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Vasilyeva</surname><given-names>A. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Васильева Анастасия Андреевна, клинический ординатор</p><p>190013, Санкт-Петербург, ул. Бронницкая, д. 9</p></bio><bio xml:lang="en"><p>Anastasia A. Vasilyeva, Clinical Resident, Saint Petersburg Research Institute of Ear, Throat, Nose and Speech</p><p>9, Bronnitskaya St., St Petersburg, 190013, Russia</p></bio><email xlink:type="simple">anastasiashvindina@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>Спичак</surname><given-names>Д. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Spichak</surname><given-names>D. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Спичак Дмитрий Владимирович, врач-оториноларинголог</p><p>193036,  Санкт-Петербург, Лиговский проспект, д. 8</p></bio><bio xml:lang="en"><p>Dmitry V. Spichak, Otorhinolaryngologist</p><p>8, Ligovsky Ave., St Petersburg, 193036, Russia</p></bio><email xlink:type="simple">emetrijolen@gmail.com</email><xref ref-type="aff" rid="aff-3"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Санкт-Петербургский научно-исследовательский институт уха, горла, носа и речи</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Saint Petersburg Research Institute of Ear, Throat, Nose and Speech</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Санкт-Петербургский научно-исследовательский институт уха, горла, носа и речи; &#13;
Детский городской многопрофильный клинический центр высоких медицинских технологий имени К.А. Раухфуса; &#13;
Северо-Западный государственный медицинский университет имени И.И. Мечникова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Saint Petersburg Research Institute of Ear, Throat, Nose and Speech; &#13;
Children’s City Multidisciplinary Clinical Center of High Medical Technologies named after K.A. Rauhfus; &#13;
North-Western State Medical University named after I.I. Mechnikov</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Детский городской многопрофильный клинический центр высоких медицинских технологий имени К.А. Раухфуса</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Children’s City Multidisciplinary Clinical Center of High Medical Technologies named after K.A. Rauhfus</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>20</day><month>05</month><year>2024</year></pub-date><volume>0</volume><issue>7</issue><fpage>154</fpage><lpage>159</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Андрианов А.В., Дроздова М.В., Алексеенко С.И., Васильева А.А., Спичак Д.В., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Андрианов А.В., Дроздова М.В., Алексеенко С.И., Васильева А.А., Спичак Д.В.</copyright-holder><copyright-holder xml:lang="en">Andrianov A.V., Drozdova M.V., Alekseenko S.I., Vasilyeva A.A., Spichak D.Y.</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://www.med-sovet.pro/jour/article/view/8303">https://www.med-sovet.pro/jour/article/view/8303</self-uri><abstract><p>Заболевания носа, околоносовых пазух и верхних дыхательных путей занимают лидирующее место в структуре острой патологии у детей любых возрастов. Это касается и аденотонзиллярной проблемы у детей. Онкологические заболевания у детей встречаются достаточно редко, особенно локализующиеся в лор-органах. Необходимо помнить, что под видом острой лор-патологии и аденотонзиллярной проблемы могут скрываться и достаточно редкие, в т. ч. онкологические заболевания. Жалоба на затруднение носового дыхания может быть единственным проявлением этой патологии. Задача врача выявить онкологическую патологию на максимально раннем этапе. Основными методами для проведения дифференциальной диагностики являются рентгенологическое и эндоскопическое исследования вне зависимости от возраста. Нейрофиброматоз (НФ) – наследственное злокачественное онкологическое заболевание. В этом случае нарушается формирование эктодермальных и мезодермальных систем. Относится к факоматозам. Поражается чаще кожа, нервная и костная системы. Характерно формирование развития злокачественных новообразований. Заболевание редкое, но приводит к значительной онкологической нагрузке на нервную систему более, чем любое другое неопластическое заболевание, в связи с этим опухолевые образования могут иметь различную локализацию и клинические проявления. Заболевание поражает несколько систем с кожными, неврологическими и ортопедическими проявлениями, которые приводят к инвалидизации или смерти пациента. Целью нашего исследования явилась демонстрация редкого онкологического заболевания – нейрофиброматоза, первыми проявлениями которого была манифестация в лор-органах у ребенка 6 лет с отягощенным семейным анамнезом. Интерес представленного клинического случая заключается в редкой локализации нейрофибромы с локализацией в структурах шеи с вовлечением глотки, гортани, средостения, сосудов шеи.</p></abstract><trans-abstract xml:lang="en"><p>Diseases of the nose, paranasal sinuses and upper respiratory tract occupy a leading place in the structure of acute pathology in children of all ages. This also applies to adenotonsillar problems in children. Oncological diseases in children are quite rare, especially those localized in the ENT organs. It must be remembered that under the guise of acute ENT pathology and adenotonsillar problems, quite rare diseases, including cancer, can be hidden. A complaint of difficulty in nasal breathing may be the only manifestation of this pathology. The doctor’s task is to identify cancer pathology at the earliest possible stage. The main methods for differential diagnosis are x-ray and endoscopic examination, regardless of age. Neurofibromatosis (NF) is a group of systemic diseases that are inherited. Refers to phakomatoses. Features include disturbances in the formation of systems of ectodermal and mesodermal origin. The skin, nervous and skeletal systems are most often affected. The formation of the development of malignant neoplasms is characteristic. The disease is rare but results in a greater cancer burden on the nervous system than any other neoplastic disease. In this regard, tumor formations may have different localization and clinical manifestations. The disease affects multiple systems with cutaneous, neurological and orthopedic manifestations that lead to disability or mortality of the patient. The purpose of our study was to demonstrate a rare oncological disease – neurofibromatosis, the first manifestations of which were manifestations in the ENT organs of a 6-year-old child with a family history. The interest of the presented clinical case lies in the rare localization of neurofibroma with localization in the structures of the neck with the involvement of the pharynx, larynx, mediastinum, and neck vessels.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>нейрофиброматоз</kwd><kwd>гипертрофия небных миндалин</kwd><kwd>новообразования</kwd><kwd>клинический случай</kwd><kwd>ребенок</kwd></kwd-group><kwd-group xml:lang="en"><kwd>neurofibromatosis</kwd><kwd>hypertrophy of the palatine tonsils</kwd><kwd>neoplasms</kwd><kwd>clinical case</kwd><kwd>child</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">Киселев АС, Вавилова АА. Редкие заболевания в практике поликлинического оториноларинголога. Некоторые аспекты диагностики. 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