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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="research-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Russian Journal of Oncology</journal-id><journal-title-group><journal-title xml:lang="en">Russian Journal of Oncology</journal-title><trans-title-group xml:lang="ru"><trans-title>Российский онкологический журнал</trans-title></trans-title-group></journal-title-group><issn publication-format="print">1028-9984</issn><issn publication-format="electronic">2412-9119</issn><publisher><publisher-name xml:lang="en">Eco-Vector</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">70300</article-id><article-id pub-id-type="doi">10.17816/1028-9984-2020-25-5-170-175</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Clinical investigations</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Клинические исследования</subject></subj-group><subj-group subj-group-type="article-type"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">Epidemiology and clinical course of vulvar melanoma: experience of the Republic of Bashkortostan</article-title><trans-title-group xml:lang="ru"><trans-title>Эпидемиология и клиническое течение меланомы вульвы (на примере Республики Башкортостан)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3734-2779</contrib-id><name-alternatives><name xml:lang="en"><surname>Menshikov</surname><given-names>Konstantin V.</given-names></name><name xml:lang="ru"><surname>Меньшиков</surname><given-names>Константин Викторович</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>MD, PhD, oncologist of the Department of Chemotherapy of the Republican Clinical Oncological Dispensary, 450054, Ufa, Russian Federation, Associate Professor of the Department of Oncology with courses oncology and pathological anatomy, Bashkir State Medical University, 450008, Ufa, Russian Federation</p></bio><bio xml:lang="ru"><p>к.м.н., врач-онколог отдела химиотерапии ГАУЗ «Республиканский клинический онкологический диспансер» Министерства здравоохранения Республики Башкортостан, Россия, Республика Башкортостан, г. Уфа, пр. Октября, 73/1, 450054, доцент кафедры онкологии с курсами онкологии и патологической анатомии ФГБОУ ВО «Башкирский государственный медицинский университет» Министерства здравоохранения Российской Федерации</p></bio><email>kmenshikov80@bk.ru</email><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Bashkir State Medical University</institution></aff><aff><institution xml:lang="ru">ФГБОУ ВО «Башкирский государственный медицинский университет» Министерства здравоохранения Российской Федерации</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2020-10-15" publication-format="electronic"><day>15</day><month>10</month><year>2020</year></pub-date><volume>25</volume><issue>5</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>170</fpage><lpage>175</lpage><history><date date-type="received" iso-8601-date="2021-05-06"><day>06</day><month>05</month><year>2021</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2021, Eco-Vector</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2021, ООО "Эко-Вектор"</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="en">Eco-Vector</copyright-holder><copyright-holder xml:lang="ru">ООО "Эко-Вектор"</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/" start_date="2024-05-14"/></permissions><self-uri xlink:href="https://rjonco.com/1028-9984/article/view/70300">https://rjonco.com/1028-9984/article/view/70300</self-uri><abstract xml:lang="en"><p>In 2019, melanoma affects 66.9 per 100,000 populations in the Russian Federation, which was higher than 44.5 in 2009. Vulvar melanoma is one of the rare localizations of malignant tumors. Its disease course and prevalence differ from those of skin melanoma. Given the rarity of this localization, no large studies have been published in the Russian Federation.</p> <p><bold>Objective.</bold> This study aimed to study the clinical course and prevalence of vulvar melanoma in the Republic of Bashkortostan.</p> <p><bold>Materials and methods. </bold>A retrospective analysis of the clinical course of malignant tumors of the external genital organs was carried out in 324 patients within a 5-year period. Vulvar melanoma was diagnosed in 23 cases, which accounted for 6.5% of the total number of patients with malignant neoplasms of the female external genital organs. The clinical course and medical data in 23 patients diagnosed with vulvar melanoma were evaluated. The disease stage, characteristics of the clinical course, and presence of BRAF and c-KIT mutations were assessed. In the Republic of Bashkortostan, the incidence of vulvar melanoma was estimated based on the data of the Republican Clinical Oncological Dispensary.</p> <p><bold>Results.</bold> Within 5 years, 23 patients were diagnosed with vulvar melanoma, which is a very small proportion relative the total number of patients with cancer. Difficulties in determining the scope of adequate surgical treatment and presence of prognostic factors define the prognosis of the disease. Most of the patients at the first stage of the disease received surgical treatment. The scope of surgical treatment was based on the localization of the primary tumor and the presence of metastases in regional lymph nodes.</p> <p><bold>Conclusion.</bold> Vulvar melanoma is a rare and aggressive malignant tumor. In the Republic of Bashkortostan, as well as in the Russian Federation, no register has been established for this localization. In view of the anatomical features of this zone, the tumor develops more aggressively and characterized by early lymphogenous metastasis. In patients with fairly young age, an aggressive disease course requires special attention.</p></abstract><trans-abstract xml:lang="ru"><p>Распространëнность меланомы в Российской Федерации составляла 66,9 на 100 тыс. населения в 2019 г., тогда как данный показатель в 2009 г. – 44,5. Меланома вульвы – одна из редких локализаций злокачественных опухолей в онкологии. Течение заболевания и распространённость отличаются от меланомы кожи. Ввиду редкости данной локализации каких-либо больших исследований, посвящëнных этой теме, не опубликовано в РФ.</p> <p><bold>Целью</bold><italic> </italic>нашей работы было изучение клинического течения и распространëнности меланомы вульвы в Республике Башкортостан.</p> <p><bold>Материалы и методы.</bold> Проведëн ретроспективный анализ клинического течения злокачественных опухолей наружных половых органов у 324 больных за 5-летний период. В 23 случаях был установлен диагноз «меланома вульвы», что составило 6,5% от общего числа пациенток со злокачественными новообразованиями женских наружных половых органов. Проведён анализ клинического течения, медицинских данных у 23 пациенток с диагнозом «меланома вульвы». Проведена оценка стадии заболевания, особенностей клинического течения, наличия BRAF и c-KIT мутаций. В Республике Башкортостан заболеваемость меланомой вульвы оценена по материалам работы Республиканского клинического онкологического диспансера.</p> <p><bold>Результаты.</bold> Трудности в определении объëма адекватного хирургического лечения, наличие прогностических факторов определяют прогноз заболевания. Большая часть пациенток на первым этапе получила оперативное лечение. Объëм хирургического лечения определялся в зависимости от локализации первичной опухоли и наличия метастазов в регионарные лимфоузлы.</p> <p><bold>Заключение.</bold> Меланома вульвы – достаточно редкая и агрессивная злокачественная опухоль. В Республике Башкортостан, как и в Российской Федерации, нет единого реестра по данной локализации. Ввиду анатомических особенностей данной зоны опухоль ведëт себя более агрессивно, характерно раннее лимфогенное метастазирование. Достаточно молодой возраст, агрессивное течение заболевания требуют особого внимания к этой группе пациенток.</p></trans-abstract><kwd-group xml:lang="en"><kwd>vulvar melanoma</kwd><kwd>surgical treatment</kwd><kwd>BRAF</kwd><kwd>c-KIT</kwd><kwd>vulvectomy</kwd><kwd>inguinal-femoral lymphadenectomy</kwd><kwd>sentinel lymph node</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>меланома вульвы</kwd><kwd>хирургическое лечение</kwd><kwd>BRAF</kwd><kwd>c-KIT</kwd><kwd>вульвэктомия</kwd><kwd>пахово-бедренная лимфаденэктомия</kwd><kwd>сторожевой лимфатический узел</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">Kaprin AD, red. The state of cancer care for the population of Russia in 2019. M.: MNIOI im. P.A. Gertsena; 2020;239. (In Russ).</mixed-citation><mixed-citation xml:lang="ru">Каприн А.Д., ред. Состояние онкологической помощи населению России в 2019 году. М.: МНИОИ им. 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