OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press87210.17650/1818-8346-2023-18-4-104-114NEW DIRECTIONS, DIAGNOSTIC OPPORTUNITIES, AND TREATMENT ADVANCESНОВЫЕ НАПРАВЛЕНИЯ, ВОЗМОЖНОСТИ ДИАГНОСТИКИ И УСПЕХИ ЛЕЧЕНИЯDiagnostic and therapeutical approaches to H3K27M-altered diffuse midline glioma in children: a reviewПодходы к диагностике и терапии H3K27M-измененных диффузных срединных глиом у детей: обзор литературыhttps://orcid.org/0000-0001-9540-1162MorgachevaD. A.МоргачеваД. А.
Russian Federation

Daria A. Morgacheva 

2 Akkuratova St., Saint Petersburg 197341

Дарья Андреевна Моргачева 

197341 Санкт-Петербург, ул. Аккуратова, 2

morgacheva_da@almazovcentre.ruhttps://orcid.org/0000-0001-9721-3827SitovskaiaD. A.СитовскаяД. А.
Russian Federation

12 Mayakovskogo St., Saint Petersburg 191014

191014 Санкт- Петербург, ул. Маяковского, 12

https://orcid.org/0000-0002-2003-0982DinikinaYu. V.ДиникинаЮ. В.
Russian Federation

2 Akkuratova St., Saint Petersburg 197341

197341 Санкт-Петербург, ул. Аккуратова, 2

V.A. Almazov National Medical Research Centre, Ministry of Health of RussiaФГБУ «Национальный медицинский исследовательский центр им. В.А. Алмазова» Минздрава РоссииA.L. Polenov Neurosurgical Institute – branch of V.A. Almazov National Medical Research Centre, Ministry of Health of RussiaРоссийский научно-исследовательский нейрохирургический институт им. проф. А.Л. Поленова – филиал ФГБУ «Национальный медицинский исследовательский центр им. В.А. Алмазова» Минздрава России091220231841041140812202308122023https://oncohematology.abvpress.ru/ongm/article/view/872

H3K27M-altered diffuse midline gliomas are the most devastating pediatric brain tumors. These tumors are characterized by lesion of central nervous system midline structures, diffuse infiltrative growth and fatal prognosis. The pathogenesis of H3K27M-altered diffuse midline glioma is based on unique epigenetic and genetic changes which are associated with histone 3 (H3) alterations. Clinical disease course usually is non-specific, that could hamper diagnosis establishment and defines high prevalence of disseminated tumor stages. Diagnostic approach includes neuroimaging, various laboratory and molecular methods, including high throughput sequencing, which allows finding potential targets for precise therapy. Despite the availability of anti-tumor technologies, including targeted therapy and immunotherapy, the standard of care for H3K27M-altered diffuse midline glioma is radiation therapy, which does not allow achieving long-term event-free survival. A dismal prognosis and absence of curative options for these tumors determine the necessity of new treatment methods search that could improve patients’ outcome. In this article we present current worldwide data of the diagnosis and treatment trends in H3K27M-altered diffuse midline glioma.

H3K27M-измененные диффузные срединные глиомы представляют собой наиболее агрессивный тип глиом детского возраста. эти опухоли характеризуются поражением срединных структур центральной нервной системы, диффузным инфильтративным ростом и фатальным прогнозом. В основе патогенеза H3K27M-измененных диффузных срединных глиом лежат уникальные эпигенетические и генетические изменения, ассоциированные с альтерациями вариантов гистона 3 (H3). клиническая картина неспецифична, что может затруднять своевременную постановку диагноза и определяет высокую частоту распространенных форм заболевания. В диагностике данного типа глиом используется нейровизуализация, а также различные методы лабораторной и молекулярной диагностики, включая высокопроизводительное секвенирование, что позволяет оценить наличие потенциальных мишеней для направленной терапии. Несмотря на доступность противоопухолевых технологий, включая таргетную и иммунотерапию, стандартом лечения H3K27M-измененных диффузных срединных глиом остается лучевая терапия, которая не позволяет достигать длительной бессобытийной выживаемости. Неблагоприятный прогноз и ограниченность куративных опций данного типа опухоли обусловливают необходимость поиска новых методов лечения, способных увеличить выживаемость пациентов этой группы. В статье представлены современные данные мировой литературы по диагностике и трендам в лечении H3K27M-измененных диффузных срединных глиом у детей.

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