nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2020-7-4-48-55nodgo-667Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSПримитивная миксоидная мезенхимальная опухоль младенцев. Обзор литературыPrimitive myxoid mesenchymal tumor of infancy. Review of literaturehttps://orcid.org/0000-0002-5268-8789ФоченковаВ. А.FochenkovaV. A.

Фоченкова Валерия Андреевна - врач-детский онколог отделения химиотерапии (противоопухолевой лекарственной терапии) и комбинированного лечения опухолей у детей СПб КНпЦСВМП(о).197758, Санкт-Петербург, пос. Песочный, ул. Ленинградская, 68А.

Pediatric Oncologist Department of Chemotherapy (Anticancer Drug Therapy) and Combined Treatment of Tumors in Children at the Saint Petersburg Clinical Scientific and Practical Center of Specialized Medical Assistance (Oncological).68А Leningradskaya St., Pesochny, S.-Petersburg, 197758.

valeria.fochenkova@gmai.comhttps://orcid.org/0000-0001-7276-9976РадулескуГ. Г.RaduleskuG. G.

Врач-детский онколог отделения химиотерапии (противоопухолевой лекарственной терапии) и комбинированного лечения опухолей у детей СПб КНпЦСВМП(о).197758, Санкт-Петербург, пос. Песочный, ул. Ленинградская, 68А.

Pediatric Oncologist Department of Chemotherapy (Anticancer Drug Therapy) and Combined Treatment of Tumors in Children at the Saint Petersburg Clinical Scientific and Practical Center of Specialized Medical Assistance (Oncological).68А Leningradskaya St., Pesochny, S.-Petersburg, 197758.

radulesky@mail.ruhttps://orcid.org/0000-0001-8261-2438РысевН. А.RysevN. A.

Кандидат биологических наук, научный сотрудник патологоанатомического отделения СПб КНпЦСВМП(о).197758, Санкт-Петербург, пос. Песочный, ул. Ленинградская, 68А.

Cand. of Sci. (Biol.), Senior Researcher of the Pathology Department at the Saint Petersburg Clinical Scientific and Practical Center of Specialized Medical Assistance (Oncological).68А Leningradskaya St., Pesochny, S.-Petersburg, 197758.

nikrysev@gmail.comhttps://orcid.org/0000-0002-8552-7682КлецкаяИ. С.KletskayaI. S.

Врач-патологоанатом патологоанатомического отделения НМИЦ ДГОИ им. Дмитрия Рогачева.117997, Москва, ул. Саморы Машела,1.

Pathologist of the Pathology Department at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia.1 Samory Mashela St., Moscow, 117997.

ikletskaya@gmail.comhttps://orcid.org/0000-0001-7732-8184КоноваловД. М.KonovalovD. M.

Кандидат медицинских наук, заведующий патологоанатомическим отделением НМИЦ ДГОИ им. Дмитрия Рогачева, доцент кафедры патологической анатомии РМАНПО.117997, Москва, ул. Саморы Машела,1; 125993, Москва, ул. Баррикадная, 2/1, стр. 1.

Cand. of Sci. (Med.), Head of the Pathology Department at Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia, Associate Professor of the Pathology Department at the Russian Medical Academy of Continuing Professional Education, Ministry of Health of Russia.1 Samory Mashela St., Moscow, 117997; Bld. 1, 2/1 Barrikadnaya St., Moscow, 125993.

dmk_nadf@mail.ruhttps://orcid.org/0000-0002-7471-7181БелогуроваМ. Б.BelogurovaM. B.

Доктор медицинских наук, профессор, заведующая отделением химиотерапии (противоопухолевой лекарственной терапии) и комбинированного лечения опухолей у детей СПб КНпЦСВМП(о), ведущий научный сотрудник института гематологии НМИЦ им. В.А. Алмазова.197758, Санкт-Петербург, пос. Песочный, ул. Ленинградская, 68А; 197341, Санкт-Петербург, ул. Аккуратова, 2.

Dr. of Sci. (Med.), Professor, Head of the Department of Chemotherapy (Anticancer Drug Therapy) and Combined Treatment of Tumors in Children at the Saint Petersburg Clinical Scientific and Practical Center of Specialized Medical Assistance (Oncological), Leading Scientific Collaborator of Institution of Hematology of Almazov National Medical Research Center, Ministry of Health of Russia.68А Leningradskaya St., Pesochny, S.-Petersburg, 197758; 2 Akkuratova St., S.-Petersburg, 197341.

deton.hospital31@inbox.ruСанкт-Петербургский клинический научно-практический центр специализированных видов медицинской помощи (онкологический)РоссияSaint Petersburg Clinical Scientific and Practical Center of Specialized Medical Assistance (Oncological)Russian FederationНациональный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaRussian FederationНациональный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева Минздрава России; ДПО РМАНПО Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia; Russian Medical Academy of Continuous Professional Education, Ministry of Health of RussiaRussian FederationСанкт-Петербургский клинический научно-практический центр специализированных видов медицинской помощи (онкологический); Национальный медицинский исследовательский центр имени В.А. Алмазова Минздрава РоссииРоссияSaint Petersburg Clinical Scientific and Practical Center of Specialized Medical Assistance (Oncological); Almazov National Medical Research Centre, Ministry of Health of RussiaRussian Federation202007012021744855Copyright © Фоченкова В.А., Радулеску Г.Г., Рысев Н.А., Клецкая И.С., Коновалов Д.М., Белогурова М.Б., 20212021Фоченкова В.А., Радулеску Г.Г., Рысев Н.А., Клецкая И.С., Коновалов Д.М., Белогурова М.Б.Fochenkova V.A., Radulesku G.G., Rysev N.A., Kletskaya I.S., Konovalov D.M., Belogurova M.B.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nodgo.org/jour/article/view/667

Примитивная миксоидная мезенхимальная опухоль младенцев (ПММОМ) — агрессивная опухоль с высокой частотой локальных рецидивов, редким отдаленным метастазированием, плохим ответом на химиотерапию.ПММОМ является редкой мягкотканной саркомой первого года жизни, выделенной из группы инфантильных фибросарком (ИФ) и миофибробластических опухолей. Она отличается от других опухолей клиническим течением, морфологическими чертами и в первую очередь молекулярно-генетическими изменениями. Для ИФ характерна перестройка гена ETV6-NTRK3, не выявляемая ни у одного пациента с ПММОМ. У больных ПММОМ обнаружена внутренняя тандемная дупликация BCOR (BCOR ITD), которая позволяет выделить данную опухоль из группы ИФ.В настоящее время не определены единые подходы к лечению этой опухоли. Радикальное хирургическое удаление является предпочтительным методом терапии. BCOR ITD служит терапевтической мишенью. В настоящее время идёт поиск таргетных препаратов, которые, возможно, решат проблему лечения ПММОМ.

Primitive myxoid mesenchymal tumor of infancy (PMMTI) is an aggressive tumor with a high rate of local recurrence, rare metastasis and poor response to chemotherapy.PMMTI is a rare soft-tissue sarcoma that preferentially affects infants. It is isolated from the group of infantile fibrosarcomas or myofibroblastic tumors. PMMTI differs from other tumors in the clinical course, morphological features and primarily molecular genetic changes. ETV6-NTRK3 gene fusion is characteristic of infantile fibrosarcomas. However, it is not detected in patients with PMMTI. BCOR ITD had been described in patients with PMMTI and it allowed to define this tumor from the group of infantile fibrosarcomas.Unified treatment approaches have not been identified for this tumor yet. Radical surgical removal is preferred treatment. BCOR ITD is a target. Nowadays search for targeted drugs is ongoing and it could help to solve the problem of PMMTI treatment.

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The authors declare that there are no conflicts of interest present.