Russian Journal of Pediatric SurgeryRussian Journal of Pediatric SurgeryДетская хирургия1560-95102412-0677Eco-Vector74710.55308/1560-9510-2023-27-4-261-271Original Study ArticlesОригинальные исследованияKaposiform hemangioendotheliomas and tufted angiomas: Sirolimus for treating childrenКапошиформные гемангиоэндотелиомы и пучковые ангиомы: опыт лечения сиролимусом у детейhttps://orcid.org/0000-0001-6382-8057KondrashovaZ. A.КондрашоваЗ. А.
Russian Federation

 Zinaida А. Kondrashova, MD, hematologist, department of hematology and chemiotherapy 

 117997 Moscow 

 117997, Moscow 

Кондрашова Зинаида Андреевна, врач-гематолог отделения гематологии и химиотерапии № 3 РДКБ

117997, Москва 

117997, Москва 

z.a.kondrashova@gmail.comhttps://orcid.org/0000-0002-4252-8829DonyushE. K.ДонюшЕ. К.
Russian Federation

 117997 Moscow 

 117997, Moscow 

 117997, Москва 

 117997, Москва 

https://orcid.org/0000-0003-3196-901XKorotkayaE. A.КороткаяЕ. А.
Russian Federation

 117997, Moscow 

 117997, Москва 

https://orcid.org/0000-0002-8552-7682KletskayaI. S.КлецкаяИ. С.
Russian Federation

 117997 Moscow 

 117997, Москва 

https://orcid.org/0000-0002-5287-7889GarbuzovR. V.ГарбузовР. В.
Russian Federation

 117997 Moscow 

117997, Москва

https://orcid.org/0000-0002-9554-6414PolyaevYu. A.ПоляевЮ. А.
Russian Federation

 117997 Moscow 

 117997, Москва 

https://orcid.org/0000-0003-3317-3058Myl’nikovA. A.МыльниковА. А.
Russian Federation

 117997 Moscow 

 117997, Москва 

Russian Children's Clinical Hospital of Pirogov Russian National Research Medical UniversityОбособленное структурное подразделение «Российская детская клиническая больница ФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской ФедерацииPirogov Russian National Research Medical UniversityФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской Федерации241020232742612711711202222102023Copyright ©; 2023, Russian Journal of Pediatric SurgeryCopyright ©; 2023, Детская хирургия. Журнал им. Ю.Ф. Исакова2023Russian Journal of Pediatric SurgeryДетская хирургия. Журнал им. Ю.Ф. Исаковаhttps://jps-nmp.ru/jour/article/view/747

Introduction. Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are extremely rare vascular tumors characterized by pathologically progressive angiogenesis and lymphangiogenesis. They are also associated with the development of the KazabachMerritt Phenomenon (KMP; Kasabach-Merritt Phenomenon).Material and methods. 8 patients (5 boys, 3 girls) with kaposiform hemangioendothelioma (two out of them with Kazabakh-Merritt syndrome) and 4 patients (3 boys, 1 girl) with tufted angioma were treated at the Russian Children's Clinical Hospital of Pirogov Russian National Research Medical University (Moscow, Russia) . All patients were prescribed Sirolimus therapy for antiproliferative purpose.Results. In all children, positive changes were registered: reduction of vascular tumor volume, gradual improvement of clinical picture and control of complications: pain syndrome, KMP, improvement or restoration of functions in affected organs /systems (increase/restoration of movement volume in joints, compensation for length discrepancy in lower legs). Complete resorption of vascular tumors and clinical symptoms were registered in 3 patients.Conclusion. The obtained encouraging results after Sirolimus therapy prescribed for treating KME and TA allow the authors to recommend this therapy to patients with KME with or without KMP and TA. However, further researches with a large sample of participants is required to confirm the obtained results.

Введение. Капошиформные гемангиоэндотелиомы (КГЭ) и пучковые ангиомы (ПА) – чрезвычайно редкие сосудистые опухоли, характеризующиеся патологически прогрессирующим ангиогенезом и лимфангиогенезом, и связаны с развитием синдрома (феномена) Казабаха–Мерритт (СKM).Материал и методы. В Российской детской клинической больнице ФГАОУ ВО «РНИМУ им. Н.И. Пирогова» МЗ РФ пролечены 8 пациентов (5 мальчиков и 3 девочки) с капошиформной гемангиоэндотелиомой (из них 2 ребёнка с синдромом Казабаха–Мерритт) и 4 пациента (3 мальчика, 1 девочка) с пучковой ангиомой. Все пациенты получали терапию сиролимусом с антипролиферативной целью.Результаты. У всех детей отмечено сокращение объёма сосудистой опухоли, последовательно прослеживалось улучшение клинической симптоматики и купирование осложнений: болевого синдрома, СKM, улучшение или восстановление функций поражённых органов/систем (увеличение/восстановление объёма движений в суставах, компенсация разницы длины нижних конечностей). У 3 пациентов отмечено полное разрешение сосудистой опухоли и клинической симптоматики.Заключение. Получены обнадёживающие данные, подтверждающие эффективность использования сиролимуса в лечении КГЭ и ПА, что позволяет предложить данный метод лечения в качестве первой линии терапии при КГЭ с или без СKM и ПА. Однако для подтверждения этих результатов требуется исследование при большой выборке участников.

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