nodgoРоссийский журнал детской гематологии и онкологии (РЖДГиО)Russian Journal of Pediatric Hematology and Oncology2311-12672413-5496LTD “Graphica”10.21682/2311-1267-2020-7-1-51-61nodgo-574Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSХориоидкарциномы у детей: обзор литературыChoroid plexus carcinoma: review of literaturehttps://orcid.org/0000-0002-2977-665XВалиахметоваЭ. Ф.ValiakhmetovaA. F.

Эндже Фаварисовна Валиахметова, врач-детский онколог 1-го нейрохирургического отделения НМИЦ нейрохирургии им. акад. Н.Н. Бурденко

SPIN-код: 3519-4972

125047, Москва, ул. 4-я Тверская-Ямская, 16

Pediatric Oncologist 1st Children Neurosurgical Department

SPIN-code: 3519-4972

16 4th Tverskaya-Yamskaya St., Moscow, 125047, Russia

andgeval@gmail.comhttps://orcid.org/0000-0001-7750-5216ПапушаЛ. И.PapushaL. I.

к.м.н., врач-детский онколог, старший научный сотрудник НМИЦ ДГОИ им. Дмитрия Рогачева

117997, Москва, ул. Саморы Машела, 1

Cand. of Sci. (Med.), Pediatric Oncologist, Senior Physician 

1 Samory Mashela St., Moscow, 117997, Russia

ludmila.mur@mail.ruhttps://orcid.org/0000-0003-3007-3772ЯськоЛ. А.YaskoL. A.

к.б.н., старший научный сотрудник НМИЦ ДГОИ им. Дмитрия Рогачева

117997, Москва, ул. Саморы Машела, 1

Cand. of Sci. (Biol.), Senior Researcher 

1 Samory Mashela St., Moscow, 117997, Russia

milayar@yandex.ruhttps://orcid.org/0000-0003-1308-8622ДруйА. Е.DruyA. E.

к.м.н., заведующий отделом клинической лабораторной диагностики лаборатории цитогенетики и молекулярной генетики НМИЦ ДГОИ им. Дмитрия Рогачева

117997, Москва, ул. Саморы Машела, 1

Cand. of Sci. (Med.), Head of the Department of Clinical Laboratory Diagnostics, Laboratory of Cytogenetics and Molecular Genetics 

1 Samory Mashela St., Moscow, 117997, Russia

dr-drui@yandex.ruГорелышевС. К.GorelyshevS. K.

д.м.н., профессор, заведующий 1-м детским нейрохирургическим отделением НМИЦ нейрохирургии им. акад. Н.Н. Бурденко

125047, Москва, ул. 4-я Тверская-Ямская, 16

Dr. of Sci. (Med.), Professor, Head of the 1st Children Neurosurgical Department

16 4th Tverskaya-Yamskaya St., Moscow, 125047, Russia

sgorel@nsi.ruhttps://orcid.org/0000-0002-9300-5198КарачунскийА. И.KarachunskiyA. I.

д.м.н., профессор, заместитель генерального директора – директор Института онкологии, радиологии и ядерной медицины НМИЦ ДГОИ им. Дмитрия Рогачева

117997, Москва, ул. Саморы Машела, 1; 117997, Москва, ул. Островитянова, 1

Dr. of Sci. (Med.), Professor, Deputy General Director – Director of the Institute of Oncology Radiology and Nuclear Medicine of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia

1 Samory Mashela St., Moscow, 117997, Russia; 1 Ostrovityanova St., Moscow, 117997, Russia

aikarat@mail.ruФГАУ «Национальный медицинский исследовательский центр нейрохирургии имени академика Н.Н. Бурденко» Минздрава РоссииРоссияN.N. Burdenko National Medical Research Center of Neurosurgery, Ministry of Health of RussiaRussian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии имени Дмитрия Рогачева» Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaRussian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии имени Дмитрия Рогачева» Минздрава России; ФГАОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia; N.I. Pirogov Russian National Research Medical University, Ministry of Health of RussiaRussian Federation202002032020715161Copyright © Валиахметова Э.Ф., Папуша Л.И., Ясько Л.А., Друй А.Е., Горелышев С.К., Карачунский А.И., 20202020Валиахметова Э.Ф., Папуша Л.И., Ясько Л.А., Друй А.Е., Горелышев С.К., Карачунский А.И.Valiakhmetova A.F., Papusha L.I., Yasko L.A., Druy A.E., Gorelyshev S.K., Karachunskiy A.I.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nodgo.org/jour/article/view/574

Хориоидкарцинома (ХК) – это редкая злокачественная опухоль, возникающая из эпителия сосудистого сплетения головного мозга. Более 80 % ХК встречаются у детей. Основную роль в патогенезе этих опухолей играют мутации в гене ТР53. ХК в 40 % случаев ассоциированы с синдромом Ли–Фраумени. Показатели выживаемости у пациентов с ХК и синдромом Ли–Фраумени крайне низкие. Стандарты терапии пациентов с ХК не определены. Известно, что степень хирургической резекции имеет прогностическое значение. Роль адъювантной терапии при ХК остается неясной: дозы и объемы лучевой терапии (ЛТ), комбинации химиотерапевтических препаратов, тайминг и сочетание ЛТ и химиотерапии (ХТ) не выяснены. Кроме того, на сегодняшний день нет ни стандартной схемы ХТ, ни международного проспективного исследования по оценке эффективности и токсичности различных комбинаций цитостатиков при ХК. В статье представлен обзор существующих молекулярно-генетических изменений, методов диагностики и лечения ХК.

Choroid plexus carcinoma (CPC) is a rare malignant tumor arising from the epithelium of the choroid plexus of the brain. More than 80 % of CPCs occur in children. Mutations in the TP53 gene is played the main role in the pathogenesis of these tumors. Choroid plexus carcinomas in 40 % of cases are associated with Li–Fraumeni syndrome. Survival rates in patients with CPC and Li–Fraumeni syndrome are extremely low. The standards of the therapy for patients with CPC are not defined. The extent of surgical resection and treatment modality correlate with prognosis. The role of adjuvant therapy in CPC remains unclear: doses and volumes of radiation therapy (RT), combinations of chemotherapeutic drugs, timing, and a combination of RT and chemotherapy (CT) have not been identified. Also, there is neither a standard CT regimen nor a prospective international study assessing the efficacy and toxicity of various combinations of cytostatics in patients with CPC. The article presents an overview of the existing molecular genetic changes, existing methods for the diagnosis and treatment of choroid plexus carcinoma.

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The authors declare that there are no conflicts of interest present.