Первично-множественные церебральные опухоли различных гистологических типов. эпидемиология, структура, генетические предпосылки

Актуальность. Первично-множественные церебральные опухоли различных гистологических типов представляют собой плохо изученную группу заболеваний. Развитие у одного пациента нескольких патологических образований становится клинической проблемой, т. к. подобные ситуации не регламентируются рекомендациями и врачу приходится принимать решение, опираясь лишь на собственный опыт и здравый смысл. Ситуация, при которой в организме больного развивается несколько независимых друг от друга новообразований, вызывает научный интерес и наталкивает на мысль о генетической детерминированности процессов, а также ряде типовых эпигенетических поломок, приводящих к их возникновению. В статье рассматриваются возможные причины, приводящие к одномоментному развитию менингиом, шванном черепных нервов, астроцитарных опухолей, аденом гипофиза и других, более редких церебральных опухолей, включая феномен «опухолевой коллизии». Выводы. По всей видимости, первично-множественные церебральные опухоли различных гистологических типов представляют собой нозологический кластер, включающий в себя состояния, вызванные различными по природе патогенетическими процессами. В некоторых случаях новообразования объединяет явная генетическая детерминированность процессов канцерогенеза. Многие из них связаны с наследственными опухолевыми синдромами (нейрофиброматоз 1 и 2 типов, шванноматоз; туберозный склероз; синдромы Нунан, LEOPARD, Костелло, Легиуса, Тюрко, Ли-Фраумени, DICER1, фон Гиппеля–Линдау и многие другие). В других случаях прослеживаются механизмы паракринной природы процесса. Третьи, по всей видимости, действительно являются случайными сочетаниями спорадически возникающих опухолей. 

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