Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children102410.24287/j.1024YSORKNLITERATURE REVIEWОБЗОР ЛИТЕРАТУРЫReview ArticleCurrent approaches to the treatment of Ewing sarcoma in children and adolescentsСовременные подходы к лечению пациентов детского и подросткового возраста с саркомой Юингаhttps://orcid.org/0000-0001-6296-4305VilesovaIrina G.ВилесоваИрина Григорьевна
Russian Federation

a pediatric oncologist at the Short-Term Treatment Facility

врач-детский онколог стационара кратковременного лечения

galanthus@mail.ruhttps://orcid.org/0000-0002-9846-2793SalnikovaE. A.СальниковаЕ. А.
Russian Federation
galanthus@mail.ruhttps://orcid.org/0000-0002-2057-2036PshonkinA. V.ПшонкинА. В.
Russian Federation
galanthus@mail.ruhttps://orcid.org/0000-0002-6848-8714KonoplevaE. I.КоноплеваЕ. И.
Russian Federation
galanthus@mail.ruhttps://orcid.org/0000-0002-9300-198XKarachunskiyA. I.КарачунскийА. И.
Russian Federation
galanthus@mail.ruThe Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России25092025131220252431871941509202517092025Copyright ©; 2025, «D. Rogachev NMRCPHOI»Copyright ©; 2025, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2025«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/1024

Ewing sarcoma (ES) is a rare and aggressive malignant tumor that mostly affects the bones. It is characterized by a translocation fusing a member of the FET family and one of the genes of the ETS family. The tumor requires multimodality treatment that includes chemotherapy and adequate local control (surgery and/or radiation therapy). Here, our goal was to report on current treatment approaches for ES in children and adolescents. Presently, an intensified VDC (vincristine, doxorubicin, сyclophosphamide)/IE (ifosfamide, etoposide) regimen with cycles given every 14 days is the preferred neoadjuvant chemotherapy option. Highdose chemotherapy was found to be effective in the treatment of patients with high-risk localized ES. In disseminated ES, this treatment is ineffective and hence is not used. Metronomic therapy that can include both low-dose chemotherapy and targeted drugs is prescribed to patients with relapsed/refractory ES, as well as to primary patients with initially metastatic disease.

Саркома Юинга (CЮ) — редкое агрессивное злокачественное новообразование, поражающее в большинстве случаев кости и характеризующееся наличием транслокации гена семейства FET с одним из генов семейства ETS. Комплексное лечение заболевания включает химиотерапию и адекватный локальный контроль (хирургическое лечение и/или лучевая терапия). Целью данной работы является представление современных подходов к медикаментозной терапии пациентов детского и подросткового возраста с СЮ. На сегодняшний день предпочтительным вариантом неоадъювантной химиотерапии является интенсифицированная схема VDC (винкристин, доксорубицин, циклофосфамид)/IE (ифосфамид, этопозид) с интервалом 14 дней. Высокодозная химиотерапия показала свою эффективность в лечении пациентов с локализованной формой СЮ из группы высокого риска. При диссеминированной форме заболевания ввиду неэффективности данный вариант лечения не проводится. Метрономная терапия может включать как низкодозовую химиотерапию, так и препараты направленного действия, и назначается пациентам с рефрактерной, рецидивирующей СЮ, а также первичным больным с исходно метастатической формой заболевания.

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