Bone and soft tissue sarcomas, tumors of the skinBone and soft tissue sarcomas, tumors of the skinСаркомы костей, мягких тканей и опухоли кожи2219-46142782-3687Publishing House ABV Press242ArticlesСтатьиMODERN APPROACHES IN THE TREATMENT OF PATIENTS WITH RHABDOMYOSARCOMA OF THE URINOGENITAL SYSTEMСовременные подходы в лечении детей с рабдомиосаркомой мочеполовой системыРябовА. Б.
Russian Federation
ryabovdoc@mail.ruФигуринК. М.
Russian Federation
НечушкинаИ. В.
Russian Federation
ЖорданияК. И.
Russian Federation
КапковаО. А.
Russian Federation
КазанцевА. П.
Russian Federation
КеримовП. А.
Russian Federation
РубанскийМ. А.
Russian Federation
РыбаковаД. В.
Russian Federation
ВолковаМ. А.
Russian Federation
МатвеевВ. Б.
Russian Federation
ИвановаН. М.
Russian Federation
ПоляковВ. Г.
Russian Federation
RyabovA. B.
Russian Federation
FigurinK. M.
Russian Federation
NechushkinaI. V.
Russian Federation
JordanyaK. I.
Russian Federation
KapkovaO. A.
Russian Federation
KazantsevA. P.
Russian Federation
KerimovP. A.
Russian Federation
RubanskiyM. A.
Russian Federation
RybakovaD. V.
Russian Federation
VolkovaM. A.
Russian Federation
MatveevV. B.
Russian Federation
IvanovaN. M.
Russian Federation
PolyakovV. G.
Russian Federation
НИИ ДОГ РОНЦ им. Н.Н.Блохина РАМНInstitute of pediatric oncology and hematology N.N. Blokhin cancer research center RAMS1107201133576511012022Copyright ©; 2011, ., ., ., ., ., ., ., ., ., ., ., ., ., Ryabov A.B., Figurin K.M., Nechushkina I.V., Jordanya K.I., Kapkova O.A., Kazantsev A.P., Kerimov P.A., Rubanskiy M.A., Rybakova D.V., Volkova M.A., Matveev V.B., Ivanova N.M., Polyakov V.G.Copyright ©; 2011, Рябов А.Б., Фигурин К.М., Нечушкина И.В., Жордания К.И., Капкова О.А., Казанцев А.П., Керимов П.А., Рубанский М.А., Рыбакова Д.В., Волкова М.А., Матвеев В.Б., Иванова Н.М., Поляков В.Г., Ryabov A., Figurin K., Nechushkina I., Jordanya K., Kapkova O., Kazantsev A., Kerimov P., Rubanskiy M., Rybakova D., Volkova M., Matveev V., Ivanova N., Polyakov V.2011., ., ., ., ., ., ., ., ., ., ., ., ., Ryabov A.B., Figurin K.M., Nechushkina I.V., Jordanya K.I., Kapkova O.A., Kazantsev A.P., Kerimov P.A., Rubanskiy M.A., Rybakova D.V., Volkova M.A., Matveev V.B., Ivanova N.M., Polyakov V.G.Рябов А.Б., Фигурин К.М., Нечушкина И.В., Жордания К.И., Капкова О.А., Казанцев А.П., Керимов П.А., Рубанский М.А., Рыбакова Д.В., Волкова М.А., Матвеев В.Б., Иванова Н.М., Поляков В.Г., Ryabov A., Figurin K., Nechushkina I., Jordanya K., Kapkova O., Kazantsev A., Kerimov P., Rubanskiy M., Rybakova D., Volkova M., Matveev V., Ivanova N., Polyakov V.https://creativecommons.org/licenses/by/4.0https://sarbon.abvpress.ru/jour/article/view/242

The modern approaches in the treatment of patients with rhabdomyosarcoma of the urinogenital system are discussed. Rhabdomyosarcoma (RMS) of the urinogenital system has benefited from near 30 years in more than 70% overall 5-year survival. Substantial progress has been made as a result of multi-center collaborative trials. Multimodahty treatment is based on risk stratification according to pretreatment stage, postoperative group, histology and site. Equivalent overall survival rates were reported in the last COG and SIOP trials, with worse event-free survival rates for bladder/prostate RMS in SIOP trials. The use of radiotherapy for local control was the main difference between COG and SIOP trials. Surgery is used to diagnose RMS and for local control after chemotherapy. One of the main tasks of treatment strategy is organ sparing result. Future combined studies are required to further treatment development of this childhood malignancy.

Органы мочеполовой системы (мочевой пузырь/простата, уретра, влагалище, вульва, матка, паратестикулярная зона) среди всех локализаций рабдомиосарком (РМС) поражаются в 20-26% наблюдений. На основе стратификации пациентов на группы риска разработана риск-адаптивная терапия, которая позволила у 80% пациентов в прогностически благоприятных группах обеспечить длительную выживаемость и сократить число калечащих операций: число цистэктомий уменьшилось с 78% впротоколе IRS-I (1972-1972) до 9% в IRS-IV (1993-1997); число гистерэктомий также уменьшилось с 48% в исследованиях IRS-I/II до 22% в IRS-III/IV. Общая выживаемость детей с РМС мочеполовой системы в исследованиях COG и SIOP не отличается, однако безрецидивная выживаемость в протоколе SIOP при РМС мочевого пузыря/простаты была хуже, что связано с более сдержанным отношением к применению лучевой терапии. Остаются нерешенные вопросы времени second-look операций, режимов лучевой терапии, лечения рецидивов и первично диссеминированных форм. Кроме того, достигнуто плато в результатах лечения на стандартных режимах терапии (VAC, IVA). Большие надежды связывают с применением новых препаратов при РМС (иринотекан), в том числе и таргетного действия. Разрабатываются технологии брахи- и внутриполостной лучевой терапии

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