Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children74110.24287/1726-1708-2023-22-4-23-36ORIGINAL ARTICLESОРИГИНАЛЬНЫЕ СТАТЬИAngiosarcoma in children: the experience of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology and literature reviewАнгиосаркома у детей: опыт НМИЦ ДГОИ им. Дмитрия Рогачева и обзор литературыhttps://orcid.org/0009-0000-2670-547XZatsarinnayaO. S.ЗацариннаяО. С.
Russian Federation

Olga S. Zatsarinnaya, a pediatric oncologist at the Department of Clinical  Oncology

1 Samory Mashela St., Moscow 117997

Зацаринная Ольга Сергеевна, врач-детский онколог отделения клинической онкологии

117997, Москва, ул. Саморы Машела, 1

clinoncology@gmail.comhttps://orcid.org/0009-0002-3851-6064ToporkovM. A.ТопорковМ. А.

Moscow

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mialto22@gmail.comhttps://orcid.org/0000-0001-5626-218XAndreevaN. A.АндрееваН. А.

Moscow

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andreeva793@bk.ruhttps://orcid.org/0000-0003-4042-0125TeleshovaM. V.ТелешоваМ. В.

Moscow

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teleshova_m@mail.ruhttps://orcid.org/0000-0001-7479-0007UtalievaD. T.УталиеваД. Т.

Moscow

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clinoncology@gmail.comhttps://orcid.org/0000-0003-0621-191XStradomskayaT. V.СтрадомскаяТ. В.

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stv-sergeeva@mail.ruhttps://orcid.org/0000-0001-8129-0545ShcherbakovA. P.ЩербаковА. П.

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Alexey.Shcherbakov@fccho-moscow.ruhttps://orcid.org/0000-0002-4451-3233GrachevN. S.ГрачевН. С.

Moscow

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nick-grachev@yandex.ruhttps://orcid.org/0000-0001-6313-6712BolshakovN. A.БольшаковН. А.

Moscow

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bolshakovn@yandex.ruhttps://orcid.org/0000-0001-8578-6572SidorovI. V.СидоровИ. В.

Moscow

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ilya93sidorov@gmail.comhttps://orcid.org/0000-0001-7732-8184KonovalovD. M.КоноваловД. М.

Moscow

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dmitry.konovalov@fccho-moscow.ruhttps://orcid.org/0000-0002-2537-6157NechesnyukA. V.НечеснюкА. В.

Moscow

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Alexey.Nechesnyuk@fccho-moscow.ruhttps://orcid.org/0000-0002-3767-4477ShamanskayaT. V.ШаманскаяТ. В.

Moscow

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shamanskaya.tatyana@gmail.comhttps://orcid.org/0000-0002-9054-5068ZhukovN. V.ЖуковН. В.

Moscow

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zhukov.nikolay@rambler.ruhttps://orcid.org/0000-0002-3704-8783KachanovD. Yu.КачановД. Ю.

Moscow

Москва

Denis.Kachanov@fccho-moscow.ruDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России2012202322423361906202303072023Copyright ©; 2023, «D. Rogachev NMRCPHOI»Copyright ©; 2023, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2023«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/741

Angiosarcoma is a rare and highly malignant endothelial tumor, which occurs mainly in adults and is extremely rare in children and adolescents. The German guidelines for the treatment of soft tissue sarcomas provided by the Cooperative Weichteilsarkom Studiengruppe in 2012 included a separate therapeutic regimen for pediatric angiosarcoma consisting of a combination of vincristine/doxorubicin/cyclophosphamide (VDC) and paclitaxel. The aim of this article was to describe the epidemiological, clinical, and morphological characteristics of angiosarcoma, to review treatment approaches, and to present the experience of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation in the treatment of children with angiosarcoma. Our study was approved by the Independent Ethics Committee and the Scientific Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthcare of the Russian Federation. The study included 5 patients diagnosed with angiosarcoma who had received treatment between 2012 and 2022. The patients’ parents gave consent to the use of their child's data, including photographs, for research purposes and in publications. Data on the patients’ age, gender, tumor location and extension, performed treatment and outcomes of the disease were analyzed. The male:female ratio was 0.66:1 and the median age was 5.2 years. Tumors were located in the soft tissues (n = 3) and in the bones (n = 2). In all the cases, invasion of the surrounding tissues was observed. There were no distant metastases at the time of diagnosis. Two (40%) patients underwent primary resection (R1) and 3 (60%) patients had tumor biopsy. Four patients received therapy according to the German guidelines for the treatment of angiosarcoma (VDC/paclitaxel courses), and 1 patient received treatment according to the guidelines for the management of non-rhabdomyosarcoma soft-tissue sarcomas (courses with vincristine, ifosfamide, doxorubicin/ vincristine, ifosfamide, actinomycin D). Objective response to treatment was achieved in 3 (60%) cases. Local control treatment of these 3 patients consisted of radiation therapy with a total dose of 50.4 Gy after R1 resection in 2 cases and biopsy in 1 case. After a median follow-up of 32 months, 2 patients who had received VDC/paclitaxel were alive without events (with complete and partial response), 3 patients died of progressive disease. Our data confirm the aggressive behavior of angiosarcoma in children. Protocol therapy that includes multiagent chemotherapy based on paclitaxel and doxorubicin along with local control treatment makes it possible to achieve a long-term control of the disease in some patients. However, further research on molecular and genetic characteristics of angiosarcoma is required to find potential novel targets for molecular targeted therapy. Further studies investigating the effectiveness of checkpoint inhibitors in angiosarcoma are also needed.

Ангиосаркома – редкая высокозлокачественная эндотелиальная опухоль, встречающаяся преимущественно у взрослых и крайне редко у детей и подростков. В рекомендациях по лечению мягкотканных сарком Немецкой группы (Cooperative Weichteilsarkom Study) версии 2012 г. была выделена отдельная терапевтическая ветвь для ангиосаркомы у детей, включающая комбинацию винкристина/доксорубицина/циклофосфамида (VDC) в сочетании с паклитакселом. Целью данной публикации было описание эпидемиологических, клинических и морфологических характеристик опухоли, подходов к терапии, а также представление собственного опыта ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России в лечении ангиосаркомы у детей. Настоящее исследование одобрено независимым этическим комитетом и утверждено решением ученого совета НМИЦ ДГОИ им. Дмитрия Рогачева. В исследование включены 5 пациентов с ангиосаркомой, получавших лечение в период с 2012 по 2022 г. Родители пациентов дали согласие на использование информации, в том числе фотографий детей, в научных исследованиях и публикациях. Проанализированы возраст, пол пациентов, локализация и распространенность опухолевого процесса, проведенное лечение и исходы заболевания. Соотношение мальчики:девочки – 0,66:1. Медиана возраста – 5,2 года. Опухоль располагалась в мягких тканях у 3 пациентов и в костях скелета – у 2. Во всех случаях отмечалась инвазия в окружающие ткани. Отдаленных метастазов на момент диагностики зафиксировано не было. Первичная резекция опухоли проведена 2 (40%) пациентам в объеме R1, остальным (n = 3, 60%) выполнена биопсия образования. Четырем пациентам терапия проводилась в соответствии с немецкими рекомендациями по лечению ангиосаркомы (курсы VDC/паклитаксел), 1 – по рекомендациям для нерабдомиосаркомных сарком (курсы винкристин, ифосфамид, доксорубицин/винкристин, ифосфамид, актиномицин Д). Ответ на проводимое специфическое лечение был зафиксирован у 3 (60%) пациентов. В качестве этапа локального контроля им проведена лучевая терапия в суммарной очаговой дозе 50,4 Гр: после R1-резекции в 2 случаях и после биопсии – в 1. С медианой наблюдения 32 мес 2 пациента, получивших терапию курсами VDC/ паклитаксел, живы без событий (c полным и частичным ответом), 3 – погибли от прогрессирования опухолевого процесса. Наши данные подтверждают агрессивное течение ангиосаркомы у детей. Проведение программной терапии, включающей полихимиотерапию на основе паклитаксела и доксорубицина в сочетании с локальным контролем, позволяет добиться длительного контроля заболевания у части пациентов. Однако необходимо дальнейшее исследование молекулярногенетических характеристик опухоли в целях поиска мишеней для молекулярно-направленной терапии, а также изучение эффективности ингибиторов контрольных точек при ангиосаркоме.

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