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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">endoserg</journal-id><journal-title-group><journal-title xml:lang="ru">Эндокринная хирургия</journal-title><trans-title-group xml:lang="en"><trans-title>Endocrine Surgery</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2306-3513</issn><issn pub-type="epub">2310-3965</issn><publisher><publisher-name>Типография «Печатных дел Мастер»</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.14341/serg2016413-19</article-id><article-id custom-type="elpub" pub-id-type="custom">endoserg-8323</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>Клиническая практика</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>Clinical practice</subject></subj-group></article-categories><title-group><article-title>Индолентное течение адренокортикального рака: клинико-морфологическая характеристика 7 больных.</article-title><trans-title-group xml:lang="en"><trans-title>Indolent form of adrenocortical carcinoma: clinico-morphological features of 7 patients.</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-9066-5190</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Бохян</surname><given-names>Ваган Юрикович</given-names></name><name name-style="western" xml:lang="en"><surname>Bokhian</surname><given-names>Vagan U.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., старший научный сотрудник отделения абдоминальной онкологии ФГБУ Российский онкологический научный центр им. Н.Н.Блохина Министерства здравоохранения Российской Федерации</p></bio><bio xml:lang="en"><p>MD, PhD</p></bio><email xlink:type="simple">dr_vahan@yahoo.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6300-0062</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Павловская</surname><given-names>Алентина Ивановна</given-names></name><name name-style="western" xml:lang="en"><surname>Pavlovskaya</surname><given-names>Alentina I.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., ведущий научный сотрудник отделения патанатомии опухолей человека ФГБУ Российский онкологический научный центр им. Н.Н.Блохина Министерства здравоохранения Российской Федерации</p></bio><bio xml:lang="en"><p>MD, PhD</p></bio><email xlink:type="simple">apavla-2012@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/0000-0002-6762-9511</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Коломейцева</surname><given-names>Алина Андреевна</given-names></name><name name-style="western" xml:lang="en"><surname>Kolomeytseva</surname><given-names>Alina A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к.м.н., старший научный сотрудник отделения химиотерапии ФГБУ Российский онкологический научный центр им. Н.Н.Блохина Министерства здравоохранения Российской Федерации. </p></bio><bio xml:lang="en"><p>MD, PhD</p></bio><email xlink:type="simple">almed2002@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/0000-0002-2410-7801</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Бугаёв</surname><given-names>Владислав Евгеньевич</given-names></name><name name-style="western" xml:lang="en"><surname>Bugaev</surname><given-names>Vladislav E.</given-names></name></name-alternatives><bio xml:lang="ru"><p>клинический ординатор отделения абдоминальной онкологии ФГБУ Российский онкологический научный центр им. Н.Н.Блохина Министерства здравоохранения Российской Федерации</p></bio><bio xml:lang="en"><p>MD</p></bio><email xlink:type="simple">vladbugaev@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/0000-0002-5229-8203</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Стилиди</surname><given-names>Иван Сократович</given-names></name><name name-style="western" xml:lang="en"><surname>Stilidi</surname><given-names>Ivan S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д.м.н., чл.-корр. РАН, профессор, заведующий отделением абдоминальной онкологии ФГБУ Российский онкологический научный центр им. Н.Н.Блохина Министерства здравоохранения Российской Федерации</p></bio><bio xml:lang="en"><p>MD, PhD, Professor</p></bio><email xlink:type="simple">istilidi@front.ru</email><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>&lt;p&gt;ФГБУ &amp;ldquo;Российский онкологический научный центр им. Н.Н. Блохина&amp;rdquo; Минздрава России&lt;/p&gt;</institution><country>Россия</country></aff><aff xml:lang="en"><institution>&lt;p&gt;N.N. Blokhin Russian Cancer Research Center&lt;/p&gt;</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>&lt;p&gt;ФГБУ Российский онкологический научный центр им. Н.Н. Блохина Министерства здравоохранения Российской Федерации&lt;/p&gt;</institution><country>Россия</country></aff><aff xml:lang="en"><institution>&lt;p&gt;N.N. Blokhin Russian Cancer Research Center&lt;/p&gt;</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2016</year></pub-date><pub-date pub-type="epub"><day>24</day><month>12</month><year>2016</year></pub-date><volume>10</volume><issue>4</issue><fpage>13</fpage><lpage>19</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Бохян В.Ю., Павловская А.И., Коломейцева А.А., Бугаёв В.Е., Стилиди И.С., 2016</copyright-statement><copyright-year>2016</copyright-year><copyright-holder xml:lang="ru">Бохян В.Ю., Павловская А.И., Коломейцева А.А., Бугаёв В.Е., Стилиди И.С.</copyright-holder><copyright-holder xml:lang="en">Bokhian V.U., Pavlovskaya A.I., Kolomeytseva A.A., Bugaev V.E., Stilidi I.S.</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.surg-endojournals.ru/jour/article/view/8323">https://www.surg-endojournals.ru/jour/article/view/8323</self-uri><abstract><p>Адренокортикальный рак (АКР) имеет высокий злокачественный потенциал в преобладающем большинстве случаев, однако у некоторых больных АКР характеризуется вялым, неагрессивным течением, когда пациенты с доказанными отдаленными метастазами или рецидивом могут жить без прогрессирования достаточно продолжительное время. Индолентный курс АКР определен как отсутствие роста доказанных злокачественных опухолевых узлов за период 12 мес. и более. Проведен сравнительный анализ клинико-морфологических характеристик 7 больных с индолентным АКР в сравнении с контрольной группой, состоящей из 157 больных, оперированных по поводу АКР. Изучены клинические характеристики, гистологические критерии Вейса, индекс пролиферативной активности Ki67, экспрессия белка р53, b-катенина. У 7 пациентов, оперированных многократно (до 5 раз), отмечено индолентное течение рецидивного или метастатического АКР. Максимальный срок наблюдения, когда отсутствовал рост опухоли, составил более 37 мес. Клинические характеристики больных обеих групп достоверно не отличались. Гистологические критерии Вейса, индекс Ki67, экспрессия белка р53 также достоверно не отличались. Экспрессия b-катенина, как ядерная, так и цитоплазматическая, отсутствовала в клетках АКР с индолентным течением, различия по сравнению с контрольной группой достоверны. Индолентное течение обусловлено биологическими особенностями опухоли, не отражается в клинических показателях, характеризующих первичную опухоль. Возможно, одним из механизмов, приводящих к подобному течению заболевания, является инактивация сигнального пути Wnt/b-катенин. Феномен индолентного течения АКР, характеризующегося, как правило, высоким злокачественным потенциалом, требует дальнейших исследований.</p></abstract><trans-abstract xml:lang="en"><p>Adrenocortical carcinomas (ACC) usually tends to be highly aggressive tumor with very poor prognosis, but some patients may have more indolent form of disease with much longer progression-free period despite of presence of distant metastases or repeated recurrence of disease. Stable tumor size (no growth of recurrent tumor or distant metastases for more than 12 months) was considered as indolent form of disease. We have analyzed clinical and histological characteristics (Weiss criteria, Ki-67 index, expression of p53, b-catenin) of recurrent or metastatic ACCs in 7 patients with indolent disease, and have compared it with control group consisting of 157 patients with ACC, who have undergone surgical treatment. Seven patients underwent repeated surgical treatment (up to 5 times) because of indolent form of recurrent or metastatic ACC. Longest time to progression was 37 months. There were no significant differences in clinical characteristics, Weiss score, Ki-67 index and expression level of p53 between two groups of patients. Indolent form of ACC was characterized by the absence of both nuclear and cytoplasmic expression of b-catenin expression; differences between two groups were statistically significant. Biological and molecular features of primary tumor, which usually does not correlate with clinical features, determine indolent form of ACC. Probably, inactivation of Wnt/b-catenin signaling pathway is one of the main mechanism leading to less aggressive disease progression. The phenomenon of unusually low aggressive natural history of ACC and its  biological basis needs to be further investigated.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>адренокортикальный рак</kwd><kwd>рак коры надпочечников</kwd><kwd>индолентное течение</kwd><kwd>бета-катенин</kwd><kwd>рецидив</kwd></kwd-group><kwd-group xml:lang="en"><kwd>adrenocortical carcinomas</kwd><kwd>indolent course</kwd><kwd>b-catenin</kwd><kwd>recurrent disease</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">Diolombi ML, Cheng L, Argani P, Epstein JI. Do clear cell papillary renal cell carcinomas have malignant potential? 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