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 Press244ArticlesСтатьиLIFRAUMENI SYNDROME (CASE REPORT)Синдром Ли-Фраумени (клинический случай)ЛюбченкоЛ. М.
Russian Federation
clingen@mail.ruПоспеховаН. И.
Russian Federation
СемьянихинаА. В.
Russian Federation
КарпухинА. В.
Russian Federation
ФуР. Т.
Russian Federation
ПрозоренкоЕ. В.
Russian Federation
ВолобуевА. В.
Russian Federation
СоколовскийВ. А.
Russian Federation
LyubchenkoL. N.
Russian Federation
PospekhovaN. I.
Russian Federation
SemyanikhinaA. V.
Russian Federation
KarpukhinA. V.
Russian Federation
FuR. G.
Russian Federation
ProzorenkoE. V.
Russian Federation
VolobuevA. V.
Russian Federation
SokolovskyV. A.
Russian Federation
Российский онкологический научный центр им. Н.Н. Блохина РАМНN.N. Blokhin Russian Cancer Research Center, Russian Academy of Medical SciencesМГНЦ РАМНMGRC RAMS1107201133757911012022Copyright ©; 2011, ., ., ., ., ., ., ., ., Lyubchenko L.N., Pospekhova N.I., Semyanikhina A.V., Karpukhin A.V., Fu R.G., Prozorenko E.V., Volobuev A.V., Sokolovsky V.A.Copyright ©; 2011, Любченко Л.М., Поспехова Н.И., Семьянихина А.В., Карпухин А.В., Фу Р.Т., Прозоренко Е.В., Волобуев А.В., Соколовский В.А., Lyubchenko L., Pospekhova N., Semyanikhina A., Karpukhin A., Fu R., Prozorenko E., Volobuev A., Sokolovsky V.2011., ., ., ., ., ., ., ., Lyubchenko L.N., Pospekhova N.I., Semyanikhina A.V., Karpukhin A.V., Fu R.G., Prozorenko E.V., Volobuev A.V., Sokolovsky V.A.Любченко Л.М., Поспехова Н.И., Семьянихина А.В., Карпухин А.В., Фу Р.Т., Прозоренко Е.В., Волобуев А.В., Соколовский В.А., Lyubchenko L., Pospekhova N., Semyanikhina A., Karpukhin A., Fu R., Prozorenko E., Volobuev A., Sokolovsky V.https://creativecommons.org/licenses/by/4.0https://sarbon.abvpress.ru/jour/article/view/244

The proper clinic observation of LFS (Sarcoma Family Syndrome, OMIM 151623) is considered in the present article, on the example of Russian Blokhin Cancer Reseach Center medical practice. LFS, though being heavily under researched and rarely diagnosed, is one of the most prominent syndromes among hereditary oncological pathologies. LFS - patients often don't receive necessary medical and genetic service in our country while there are accurately defined criteria of diagnostics and a series of definite recommendations on LFS - patients' management and giving medical treatment and consultation to them and their families in the world medical practice. Clinical data of LFS patients proves the necessity of multidisciplinary approaches in the management of sarcoma patients

Приводится собственное клиническое наблюдение синдрома Ли-Фраумени (Sarcoma Family Syndrome, OMIM151623) из практики РОНЦ им. Н.Н. Блохина. Оставаясь малоизученным и редко диагностируемым в нашей стране, синдром Ли-Фраумени (ЛФС) является одним из значимых среди наследственной онкологической патологии. Нередко пациенты с ЛФС не получают должной медико-генетической помощи. В мировой практике четко определены критерии постановки диагноза и дан ряд определенных рекомендаций по ведению пациентов с ЛФС, оказанию им и их семьям лечебной и консультативной помощи. Клинические данные пациентов с ЛФС обосновывают необходимость мультидисциплинарного подхода при ведении пациентов с саркомами

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