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 Press496SOFT TISSUE SARCOMASСАРКОМЫ МЯГКИХ ТКАНЕЙAngiosarcomasАнгиосаркомыFedenkoA. A.ФеденкоАлександр Александрович
Russian Federation
fedenko@eesg.ruKonevA. A.КоневА. А.
Russian Federation
AnurovaO. A.АнуроваО. А.
Russian Federation
GorbunovaV. A.ГорбуноваВ. А.
Russian Federation
SobolevskyV. A.СоболевскийВ. А.
Russian Federation
BohyanB. U.БохянБ. Ю.
Russian Federation
LyubchenkoL. N.ЛюбченкоЛ. Н.
Russian Federation
KharatishviliT. K.ХаратишвилиТ. К.
Russian Federation
FGBU N.N. Blokhin Russian Cancer Research CenterФГБУ «Российский онкологический научный центр им. Н.Н. Блохина» РАМН0302201351244103022022Copyright ©; 2013, Fedenko A.A., Konev A.A., Anurova O.A., Gorbunova V.A., Sobolevsky V.A., Bohyan B.U., Lyubchenko L.N., Kharatishvili T.K.Copyright ©; 2013, Феденко А.А., Конев А.А., Анурова О.А., Горбунова В.А., Соболевский В.А., Бохян Б.Ю., Любченко Л.Н., Харатишвили Т.К.2013Fedenko A.A., Konev A.A., Anurova O.A., Gorbunova V.A., Sobolevsky V.A., Bohyan B.U., Lyubchenko L.N., Kharatishvili T.K.Феденко А.А., Конев А.А., Анурова О.А., Горбунова В.А., Соболевский В.А., Бохян Б.Ю., Любченко Л.Н., Харатишвили Т.К.https://creativecommons.org/licenses/by/4.0https://sarbon.abvpress.ru/jour/article/view/496

Angiosarcomas are one of the rarest forms of soft tissue neoplasms. They make up a small fraction of all vascular tumors, and less than 1% of all sarcomas. Prognosis and treatment depends on the location of the primary tumor. Angiosarcomas can be divided into several clinical subgroups: angiosarcomasnot associated with lymphedema; angiosarcomas associated with lymphedema (so-called lymphangiosarcomas); angiosarcomas of the breast; angiosarcomas of deep soft tissue and radiation-induced angiosarcomas. The main treatment is radical surgery (with histological examination of surgical margins). Postoperative radiotherapy improves overall survival. There are still no recommended standardsof chemotherapy in the treatment of angiosarcomas. Taxane-based chemotherapy is possible. We recommend chemotherapy regime GemTax as 1st-line treatment, which has shown to be effective. The overall efficiency was 38%. Control of tumor growth was equal to 93%. In case of affection of the scalp weekly administration of paclitaxel is possible. In case of organ angiosarcomas a combination of ifosfamide with doxorubicin can be also used.

Ангиосаркомы одна из самых редких форм новообразований мягких тканей. Они составляют малую долю всех сосудистых опухолей и менее 1% всех сарком. Прогноз и лечение зависят от локализации первичной опухоли. Ангиосаркомы могут быть разделены на несколько клинических групп: ангиосаркома, не связанная с лимфостазом; ангиосаркома, связанная с лимфостазом (так называемая лимфангиосаркома); ангиосаркома молочной железы; ангиосаркома глубоких мягких тканей и радиоиндуцированная ангиосаркома. Основным методом лечения ангиосарком является радикальная операция (с гистологическим исследованием краев резекции). Проведение послеоперационной лучевой терапии увеличивает общую выживаемость. Рекомендованных стандартов химиотерапии в лечении ангиосарком до сих пор не существует. Возможно проведение химиотерапии на основе таксанов. Мы рекомендуем проводить химиотерапию по схеме GemTax в качестве 1-й линии лечения, которая показала свою эффективность. Общая эффективность составила 38%. Контроль роста опухоли был равен 93%. При поражении кожи волосистой части головы возможно еженедельное введение паклитаксела. При органных ангиосаркомах также может быть использована комбинация ифосфамида с доксорубицином.

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