nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2023-10-3-63-69nodgo-964Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSНейробластомы очень высокого прогностического риска: гистологические, иммунофенотипические и генетические характеристики. Обзор литературы и собственные наблюденияExtremely high prognostic risk group of neuroblastic tumors: histological, immunophenotypic and genetic characteristics. Literature review and own observationshttps://orcid.org/0000-0002-9496-3136ТаракановаА. В.TarakanovaA. V.

врач-патологоанатом отделения патологической анатомии

117997, Москва, ул. Саморы Машела, 1

Pathologist Pathological Anatomical Department

1 Samory Mashela St., Moscow, 117997

sequaciou@gmail.comhttps://orcid.org/0000-0001-5354-7067ШарлайА. С.SharlaiA. S.

врач-лабораторный генетик отделения патологической анатомии

117997, Москва, ул. Саморы Машела, 1

Laboratory Geneticist Pathological Anatomical Department

1 Samory Mashela St., Moscow, 117997

sova.vtumane@ya.ruhttps://orcid.org/0000-0003-1308-8622ДруйА. Е.DruyA. E.

к.м.н., заведующий лабораторией молекулярной онкологии

117997, Москва, ул. Саморы Машела, 1

Cand. of Sci. (Med.), Head of the Laboratory of Molecular Oncology 

1 Samory Mashela St., Moscow, 117997

alexander.druy@fccho-moscow.ruhttps://orcid.org/0000-0001-7732-8184КоноваловД. М.KonovalovD. M.

к.м.н., заведующий отделением патологической анатомии

117997, Москва, ул. Саморы Машела, 1

Cand. of Sci. (Med.), Head of the Pathology Departmen

1 Samory Mashela St., Moscow, 117997

pathmorf@mail.ruФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaRussian Federation2023301120231036369Copyright © Тараканова А.В., Шарлай А.С., Друй А.Е., Коновалов Д.М., 20232023Тараканова А.В., Шарлай А.С., Друй А.Е., Коновалов Д.М.Tarakanova A.V., Sharlai A.S., Druy A.E., Konovalov D.M.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/964

Отличительной чертой группы периферических нейробластических опухолей является их клиническая гетерогенность, варьирующая от спонтанного регресса опухоли до широко распространенного процесса, который часто устойчив к мультимодальным методам лечения. Несмотря на значительный прогресс в терапии, около 40 % пациентов с нейробластомой высокого риска умирают от рецидива заболевания при полном ответе после 1-й линии терапии. Эти 40 % считаются группой очень высокого риска, требующей интенсификации режимов терапии с момента постановки диагноза. Предметом повышенного научного и практического интереса является поиск гистологических и молекулярных предиктивных признаков этой группы для подбора корректной стратегии лечения.

Clinical heterogeneity appears to be one of the most characteristic feature of the group of peripheral neuroblastic tumors, ranging from spontaneous tumor regression to a widespread process, often resistant to multimodal therapeutic strategies. Despite significant progress in treatment, about 40 % of patients with high-risk neuroblastoma die from disease recurrence after complete response to first-line therapy. These 40 % are considered a “extremely high” risk group requiring intensification of therapeutic regimens from the time of diagnosis. Histological and molecular predictive features of this group are of high scientific and practical interest for the correct therapy.

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