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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">radiology</journal-id><journal-title-group><journal-title xml:lang="ru">Радиология — практика</journal-title><trans-title-group xml:lang="en"><trans-title>Radiology - Practice</trans-title></trans-title-group></journal-title-group><issn pub-type="epub">2713-0118</issn><publisher><publisher-name>Центральный научно-исследовательский институт лучевой диагностики</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.52560/2713-0118-2024-2-54-71</article-id><article-id custom-type="elpub" pub-id-type="custom">radiology-511</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 СТАТЬИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL RESEARCH</subject></subj-group></article-categories><title-group><article-title>Стереотаксическое облучение в лечении гемангиобластом ЦНС при болезни Гиппеля – Линдау: описание семейного случая и обзор литературы</article-title><trans-title-group xml:lang="en"><trans-title>Stereotactic Radiotherapy of VHL-Associated hemangioblastomas: A Family Case Report 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/0009-0005-1143-2030</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Лестровая</surname><given-names>А. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Lestrovaya</surname><given-names>A. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Лестровая Арина Игоревна, врач-радиотерапевт</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p><p>+7 (916) 196-65-10</p></bio><bio xml:lang="en"><p>Lestrovaya Arina Igorevna, radiotherapist</p><p>16, st. 4-ya Tverskaya-Yamskaya, Moscow, 125047</p><p>+7 (916) 196-65-10</p></bio><email xlink:type="simple">alestrovaya@nsi.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/0000-0002-4480-0275</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Пронин</surname><given-names>И. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Pronin</surname><given-names>I. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Пронин Игорь Николаевич, доктор медицинских наук, профессор, академик РАН</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p><p>+7 (916) 651-17-56</p></bio><bio xml:lang="en"><p>Pronin Igor Nikolaevich, MD, Ph. D, professor</p><p>16, st. 4-ya Tverskaya-Yamskaya, Moscow, 125047</p><p>+7 (916) 651-17-56</p></bio><email xlink:type="simple">pronin@nsi.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/0000-0002-0976-4547</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Голанов</surname><given-names>А. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Golanov</surname><given-names>A. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Голанов Андрей Владимирович, доктор медицинских наук, профессор, член-корреспондент РАН</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p><p>+7 (903) 960-87-73</p></bio><bio xml:lang="en"><p>Golanov Andrey Vladimirovich, MD, Ph. D, professor</p><p>16, st. 4-ya Tverskaya-Yamskaya, Moscow, 125047</p><p>+7 (903) 960-87-73</p></bio><email xlink:type="simple">golanov@nsi.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/0000-0002-8924-7346</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Баталов</surname><given-names>А. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Batalov</surname><given-names>A. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Баталов Артем Игоревич, кандидат медицинских наук</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p><p>+7 (916) 066-20-50</p></bio><bio xml:lang="en"><p>Batalov Artem Igorevich, Ph. D.</p><p>16, st. 4-ya Tverskaya-Yamskaya, Moscow, 125047</p><p>+7 (916) 066-20-50</p></bio><email xlink:type="simple">abatalov@nsi.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/0000-0003-3470-5802</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Антипина</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Antipina</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Антипина Наталия Анатольевна, медицинский физик</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p><p>+7 (903) 774-48-39</p></bio><bio xml:lang="en"><p>Antipina Natalia Anatolievna, medical physicist</p><p>16, st. 4-ya Tverskaya-Yamskaya, Moscow, 125047</p><p>+9 (037) 74-48-39</p></bio><email xlink:type="simple">nantipina@nsi.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/0009-0007-1614-3208</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Данилина</surname><given-names>И. И.</given-names></name><name name-style="western" xml:lang="en"><surname>Danilina</surname><given-names>I. I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Данилина Ирина Игоревна, медицинский физик</p><p>125047, Москва, ул. 4-я Тверская-Ямская, 16</p><p>+7 (915) 876-86-08</p></bio><bio xml:lang="en"><p>Danilina Irina Igorevna, medical physicist</p><p>16, st. 4-ya Tverskaya-Yamskaya, Moscow, 125047</p><p>+7 (915) 876-86-08</p></bio><email xlink:type="simple">danilinaII@nsi.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГАУ «Национальный медицинский исследовательcкий центр нейрохирургии им. ак. Н. Н. Бурденко» Минздрава России</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Burdenko National Medical Research Center of Neurosurgery of the Ministry of Health of the Russian Federation</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>27</day><month>02</month><year>2024</year></pub-date><volume>0</volume><issue>2</issue><fpage>54</fpage><lpage>71</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Лестровая А.И., Пронин И.Н., Голанов А.В., Баталов А.И., Антипина Н.А., Данилина И.И., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Лестровая А.И., Пронин И.Н., Голанов А.В., Баталов А.И., Антипина Н.А., Данилина И.И.</copyright-holder><copyright-holder xml:lang="en">Lestrovaya A.I., Pronin I.N., Golanov A.V., Batalov A.I., Antipina N.A., Danilina I.I.</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.radp.ru/jour/article/view/511">https://www.radp.ru/jour/article/view/511</self-uri><abstract><p>Болезнь Гиппеля — Линдау (БГЛ) представляет собой аутосомно-доминантный опухолевый синдром, возникающий в результате мутаций с потерей функции гена VHL и ассоциированный с опухолями различной локализации.</p><p>Гемангиобластома (ГАБ, ангиоретикулема), относительно редко встречающаяся доброкачественная богато васкуляризованная опухоль центральной нервной системы, является одним из наиболее частых проявлений и основной причиной заболеваемости и смертности пациентов с БГЛ, но может проявляться и в виде спорадической формы. Опухоль часто имеет кистозный компонент и множественный характер. В основном располагается субтенториально. МРТ — золотой стандарт диагностики, также для постановки правильного диагноза могут использоваться компьютерная томография и перфузионные методики.</p><p>Методом выбора этих опухолей является их хирургическое удаление. Показаниями к стереотаксическому облучению гемангиобластом, которые все время расширяются, являются небольшие асимптоматичные очаги, в том числе при множественном поражении, и остатки или рецидивы опухоли после их удаления. Радиохирургия проводится с высокой степенью эффективности контроля опухолевого роста, в том числе при пятилетнем и более наблюдении при низкой токсичности, что особенно важно для пациентов с минимально выраженной симптоматикой и у пациентов детского возраста, а также рекомендуется соматически отягощенным пациентам и при высоких рисках хирургического удаления этих новообразований в случае сложной локализации.</p><p>В статье описан семейный случай гемангиобластомы, прослеженный на протяжении нескольких поколений, приведен алгоритм ведения больных с синдромом БГЛ с рекомендациями по необходимому объему их диагностики, наблюдения и обсуждением подходов к их лечению.</p><p>Тщательный сбор семейного анамнеза, систематическое и правильное проведение генетического и диспансерного обследования, наблюдение за пациентами с БГЛ позволит своевременно установить правильный диагноз и более широко использовать метод стереотаксического облучения.</p></abstract><trans-abstract xml:lang="en"><p>Von Hippel-Lindau syndrome (VHL) is a rare autosomal-dominant inherited cancer syndrome with different phenotypes caused by variants in the VHL gene.</p><p>Hemangioblastoma (HAB) is a benign tumor of the central nervous system and may appear as a component of VHL disease.</p><p>HAB is one of the most common manifestations and a leading cause of morbidity and mortality in patients with BGL, but can also occur as a sporadic form. This tumor often has a cystic component and is multiple in nature. Mainly located subtentorially. MRI is the gold standard for diagnosis; computed tomography and perfusion techniques can also be used to make the correct diagnosis. The definitive treatment for these lesions is surgical resection, followed by SRS if necessary. Stereotactic irradiation is the method of choice for hemangioblastomas, especially with early detection of relatively small lesions, including those with multiple lesions. Radiosurgery is carried out with a high degree of efficiency with low toxicity, which is especially important for patients with minimally severe symptoms and in pediatric patients and also is recommended to patients with limited surgical alternatives (i.e., severe medical comorbidities, surgically unresectable tumors). SRS offered a satisfactory 5-year tumor control of HHB to VHL patients.</p><p>The article describes a family case of hemangioblastoma, traced over several generations, provides an algorithm for the management of patients with VHL syndrome with recommendations for the required scope of their diagnosis, observation and discussion of approaches to their treatment.</p><p>Careful collection of family history, genetic and follow up examination carried out systematically and correctly, monitoring of patients with VHL will allow timely establishment of the correct diagnosis and more widespread use of the stereotactic irradiation method.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>болезнь Гиппеля — Линдау</kwd><kwd>гемангиобластома</kwd><kwd>стереотаксическая радиотерапия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>Hemangioblastoma</kwd><kwd>VHL syndrome</kwd><kwd>Radiotherapy</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">Maher E. 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