nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2023-10-3-70-81nodgo-965Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL CASESВнескелетная саркома Юинга почки: описание двух редких клинических случаев и обзор мировой литературыPrimary Ewing’s sarcoma of the kidney: description of two rare clinical cases and review of world literaturehttps://orcid.org/0000-0002-2310-0106РоманцоваО. М.RomantsovaO. M.

врач-детский онколог, заведующая детским онкологическим отделением № 2 (химиотерапии опухолей опорнодвигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова

115522, Москва, Каширское шоссе, 23

Pediatric Oncologist, Head of the Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov

23 Kashirskoe Shosse, Moscow, 115522

dr.roma1986@gmail.comhttps://orcid.org/0000-0002-3822-8786ПанаринаВ. Ю.PanarinaV. Yu.

врач-детский онколог детского онкологического отделения № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова 

115522, Москва, Каширское шоссе, 23

Pediatric Oncologist Pediatric Oncology Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov

23 Kashirskoe Shosse, Moscow, 115522

veronicapanarina@yandex.ruhttps://orcid.org/0000-0003-4933-8750БолохоноваМ. А.BolokhonovaM. A.

врач-детский онколог поликлинического отделения НИИ детской онкологии и гематологии им. акад. Л.А. Дурнова

115522, Москва, Каширское шоссе, 23

Pediatric Oncologist of Polyclinic Department of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov

23 Kashirskoe Shosse, Moscow, 115522

mbolokhonova93@mail.ruhttps://orcid.org/0000-0003-0883-7801ХайрулловаВ. В.KhairullovaV. V.

врач-детский онколог детского онкологического отделения № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова

115522, Москва, Каширское шоссе, 23

Pediatric Oncologist Pediatric Oncology Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov

23 Kashirskoe Shosse, Moscow, 115522

vini999_999@mail.ruhttps://orcid.org/0000-0002-8149-254XХестановД. Б.KhestanovD. B.

к.м.н., старший научный сотрудник детского онкологического отделения № 2 (химиотерапии опухолей опорнодвигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова

115522, Москва, Каширское шоссе, 23

Cand. of Sci. (Med.), Senior Researcher of the Pediatric Oncology Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov

23 Kashirskoe Shosse, Moscow, 115522

hestanov@mail.ruhttps://orcid.org/0000-0002-1925-2457ЕфимоваМ. М.EfimovaM. M.

врач-детский онколог детского онкологического отделения № 2 (химиотерапии опухолей опорно-двигательного аппарата) НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова

115522, Москва, Каширское шоссе, 23

Pediatric Oncologist Pediatric Oncology Department No. 2 (Chemotherapy of Tumors of the Musculoskeletal System) of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov

23 Kashirskoe Shosse, Moscow, 115522

Drefimovaburgol@yandex.ruhttps://orcid.org/0000-0003-2240-069XПанфероваТ. Р.PanferovaT. R.

к.м.н., старший научный сотрудник детского отделения рентгенодиагностики консультативно-диагностического центра

115522, Москва, Каширское шоссе, 23

Cand. of Sci. (Med.), Senior Researcher of the Pediatric Radiology Department of Consultative and Diagnostic Center

23 Kashirskoe Shosse, Moscow, 115522

tizmailova@gmail.comhttps://orcid.org/0000-0003-2794-6886ИгнатенкоО. А.IgnatenkoO. A.

к.м.н., врач-рентгенолог детского отделения рентгенодиагностики консультативно-диагностического центра

115522, Москва, Каширское шоссе, 23

Cand. of Sci. (Med.), Radiologist of the Pediatric Radiology Department of Consultative and Diagnostic Center

23 Kashirskoe Shosse, Moscow, 115522

kirillovaoa@gmail.comhttps://orcid.org/0000-0003-3852-3969КозловН. А.KozlovN. A.

к.м.н., врач-патологоанатом патологоанатомического отделения отдела морфологической и молекулярно-генетической диагностики опухолей

115522, Москва, Каширское шоссе, 23

Cand. of Sci. (Med.), Pathologist Pathological Department of the Department of Morphological and Molecular-Genetic Diagnostics of Tumors 

23 Kashirskoe Shosse, Moscow, 115522

newbox13@mail.ruhttps://orcid.org/0000-0002-7297-5240СтрогановаА. М.StroganovaA. M.

к.м.н., заведующая молекулярно-биологической лабораторией отдела молекулярно-генетических исследований центра морфологической и молекулярно-генетической диагностики опухолей

115522, Москва, Каширское шоссе, 23

Cand. of Sci. (Med.), Head of the Molecular Biology Laboratory, Department of Molecular Genetic Research, Center for Morphological and Molecular Genetic Diagnostics of Tumors of the N.N. Trapeznikov Research Institute of Clinical Oncology

23 Kashirskoe Shosse, Moscow, 115522

stroganova_am@mail.ruhttps://orcid.org/0000-0002-2945-284XКиргизовК. И.KirgizovK. I.

к.м.н., заместитель директора по научной работе НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова

115522, Москва, Каширское шоссе, 23

Cand. of Sci. (Med.), Deputy Director for Scientific Work of Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov

23 Kashirskoe Shosse, Moscow, 115522

k.kirgizov@ronc.ruhttps://orcid.org/0000-0001-6131-1783ВарфоломееваС. Р.VarfolomeevaS. R.

д.м.н., профессор, директор НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова

115522, Москва, Каширское шоссе, 23

Dr. of Sci. (Med.), Professor, Director of the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov

23 Kashirskoe Shosse, Moscow, 115522

s.varfolomeeva@ronc.ruФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава РоссииРоссияN.N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of RussiaRussian Federation2023301120231037081Copyright © Романцова О.М., Панарина В.Ю., Болохонова М.А., Хайруллова В.В., Хестанов Д.Б., Ефимова М.М., Панферова Т.Р., Игнатенко О.А., Козлов Н.А., Строганова А.М., Киргизов К.И., Варфоломеева С.Р., 20232023Романцова О.М., Панарина В.Ю., Болохонова М.А., Хайруллова В.В., Хестанов Д.Б., Ефимова М.М., Панферова Т.Р., Игнатенко О.А., Козлов Н.А., Строганова А.М., Киргизов К.И., Варфоломеева С.Р.Romantsova O.M., Panarina V.Y., Bolokhonova M.A., Khairullova V.V., Khestanov D.B., Efimova M.M., Panferova T.R., Ignatenko O.A., Kozlov N.A., Stroganova A.M., Kirgizov K.I., Varfolomeeva S.R.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/965

Саркома Юинга (СЮ) почки – крайне редкая злокачественная опухоль, характеризующаяся агрессивным течением, заболевание имеет неблагоприятный прогноз. В связи с редкой встречаемостью стандарты лечения СЮ почки не разработаны, в терапии зачастую применяется комплексный подход. В настоящей статье мы предлагаем описание двух клинических случаев поражения почек СЮ у пациентов 10 и 16 лет, проходивших лечение в НИИ детской онкологии и гематологии им. акад. РАМН Л.А. Дурнова ФГБУ «НМИЦ онкологии им. Н.Н. Блохина» Минздрава России.

В первом случае имело место метастатическое поражение почки при локализации первичного очага в костях таза, во 2-м – была диагностирована первичная СЮ почки. Оба пациента на момент диагностики имели IVb стадию (согласно TNM-классификации American Joint Commission on Cancer). У обоих больных после проведения индукционной химиотерапии был получен полный терапевтический ответ (полный лечебный патоморфоз). В комплексном подходе помимо стандартной химиотерапии, хирургии и лучевой терапии была применена высокодозная химиотерапия с аутологичной трансплантацией периферических стволовых клеток, которая позволила создать благоприятные условия для долгосрочной ремиссии у одного из пациентов, даже несмотря на инициальную распространенность процесса.

Ewing’s sarcoma (ES) of the kidney is an extremely rare malignant tumor characterized by an aggressive course, and therefore the disease has an unfavorable prognosis. Due to the rare occurrence, standards for the treatment of ES of the kidney have not been developed; an integrated approach is often used in therapy. In this article, we present a description of two clinical cases of kidney damage with ES in patients aged 10 and 16 who were treated at the Research Institute of Pediatric Oncology and Hematology named after Academician of the Russian Academy of Medical Sciences L.A. Durnov at N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia. In the first case, there was a metastatic lesion of the kidney with the localization of the primary focus in the pelvic bones, in the second, primary ES of the kidney was diagnosed. Both patients had stage IVb at the time of diagnosis (TNM classification American Joint Commission on Cancer). In both patients, after induction chemotherapy, a complete therapeutic response (complete therapeutic pathomorphosis) was obtained. In an integrated approach, in addition to standard chemotherapy, surgery and radiation therapy, in both cases, high-dose chemotherapy with autologous peripheral stem cell transplantation was used, which made it possible to create favorable conditions for long-term remission in one of the patients, even despite the initial prevalence of the process.

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