oncotomskСибирский онкологический журналSiberian journal of oncology1814-48612312-3168Tomsk National Research Medical Сепtеr of the Russian Academy of Sciences10.21294/1814-4861-2021-20-1-34-45oncotomsk-1689Research ArticleКЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯCLINICAL STUDIESГЛИОМЫ ЗРИТЕЛЬНОГО ПУТИ У ДЕТЕЙ: ДИЭНЦЕФАЛЬНАЯ КАХЕКСИЯPEDIATRIC OPTIC PATHWAY GLIOMA: DIENCEPHALIC SYNDROMEhttps://orcid.org/0000-0002-2977-665XВалиахметоваЭ. Ф.ValiakhmetovaA. F.

детский онколог 1-го нейрохирургического отделения; младший научный сотрудник, отдел оптимизации терапии опухолей 

SPIN-код: 3519-4972 Россия, 125047, Москва, 4-я Тверская-Ямская улица, 16 Россия, 117997, г. Москва, ул. Саморы Машела, 1

MD, Pediatric Oncologist, 1st Children Neurosurgical Department; Junior researcher, Department of Optimization of CNS Tumor Therapy

16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia

1, Samory Machela Street, 117997, Moscow, Russia

andgeval@gmail.comМазеркинаН. А.MazerkinaN. A.

MD, DSc, Senior Researcher

16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia

https://orcid.org/0000-0001-7750-5216ПапушаЛ. И.PapushaL. I.

MD, PhD, Senior Physician, Head of the Department of Optimization of CNS Tumor Therapy

1, Samory Machela Street, 117997, Moscow, Russia

БыдановО. И.BydanovO. I.

Researcher

1, Samory Machela Street, 117997, Moscow, Russia2

ТарасоваЕ. М.TarasovaE. M.

MD, Pediatric Oncologist, Department of Hematology and Chemotherapy № 2

117, Leninsky pr., 119571, Moscow, Russia3

МедведеваО. А.MedvedevaO. A.

MD, PhD, Neurosurgeon, Children Neurosurgical Department, Junior Researcher

16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia

СероваН. К.SerovaN. K.

16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia

ЛазареваЛ. А.LazarevaL. A.

16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia

https://orcid.org/0000-0001-7045-7223ШишкинаЛ. В.ShishkinaL. V.

MD, PhD, Pathologist, Leading Specialist

16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia

ТрунинЮ. Ю.TruninYu. Yu.

16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia

НовичковаГ. А.NovichkovaG. A.

MD, DSc, Professor, General Director 

1, Samory Machela Street, 117997, Moscow, Russia

1, Ostrovityanova Street, 117997, Moscow, Russia 

ГорелышевС. К.GorelyshevS. K.

MD, DSc, Professor, Head of the 1st Children Neurosurgical Department 

16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia

1, Ostrovityanova Street, 117997, Moscow, Russia

https://orcid.org/0000-0002-9300-5198КарачунскийА. И.KarachunskyA. I.

MD, DSc, Professor, Deputy General Director

1, Samory Machela Street, 117997, Moscow, Russia

1, Ostrovityanova Street, 117997, Moscow, Russia 

ФГАУ «НМИЦ нейрохирургии им. ак. Н.Н. Бурденко» Минздрава России; ФГБУ «НМИЦ ДГОИ им. Д. Рогачева» Минздрава РоссииРоссияN.N. Burdenko National Medical Research Center of Neurosurgery, Ministry of Healthcare of Russian Federation; Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaRussian FederationФГАУ «НМИЦ нейрохирургии им. ак. Н.Н. Бурденко» Минздрава РоссииРоссияN.N. Burdenko National Medical Research Center of Neurosurgery, Ministry of Healthcare of Russian FederationRussian FederationФГБУ «НМИЦ ДГОИ им. Д. Рогачева» Минздрава РоссииРоссияDmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaRussian FederationРоссийская детская клиническая больница ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава РоссииРоссияRussian Children’s Clinical Hospital, N.I. Pirogov Russian National Research Medical UniversityRussian FederationФГАУ «НМИЦ нейрохирургии им. ак. Н.Н. Бурденко» Минздрава РоссииРоссияN.N. Burdenko National Medical Research Center of Neurosurgery, Ministry of Healthcare of Russian FederationRussian FederationФГБУ «НМИЦ ДГОИ им. Д. Рогачева» Минздрава России; ФГБОУ 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 UniversityRussian FederationФГАУ «НМИЦ нейрохирургии им. ак. Н.Н. Бурденко» Минздрава России; ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава РоссииРоссияN.N. Burdenko National Medical Research Center of Neurosurgery, Ministry of Healthcare of Russian Federation; N.I. Pirogov Russian National Research Medical UniversityRussian Federation2021030320212013445Copyright © Валиахметова Э.Ф., Мазеркина Н.А., Папуша Л.И., Быданов О.И., Тарасова Е.М., Медведева О.А., Серова Н.К., Лазарева Л.А., Шишкина Л.В., Трунин Ю.Ю., Новичкова Г.А., Горелышев С.К., Карачунский А.И., 20212021Валиахметова Э.Ф., Мазеркина Н.А., Папуша Л.И., Быданов О.И., Тарасова Е.М., Медведева О.А., Серова Н.К., Лазарева Л.А., Шишкина Л.В., Трунин Ю.Ю., Новичкова Г.А., Горелышев С.К., Карачунский А.И.Valiakhmetova A.F., Mazerkina N.A., Papusha L.I., Bydanov O.I., Tarasova E.M., Medvedeva O.A., Serova N.K., Lazareva L.A., Shishkina L.V., Trunin Y.Y., Novichkova G.A., Gorelyshev S.K., Karachunsky A.I.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.siboncoj.ru/jour/article/view/1689

Диэнцефальная кахексия (ДК) – нарушение обмена веществ, характеризующееся снижением массы тела при нормальном самочувствии и нормальном потреблении калорий. Наиболее часто данное состояние встречается при глиомах зрительного пути (ГЗП) и является фактором неблагоприятного прогноза.

Цель исследования – проанализировать особенности течения глиом зрительного пути у пациентов с ДК и без неё.

Материал и методы

Материал и методы. В исследование вошли 264 пациента в возрасте от 0 до 18 лет с первичным диагнозом «глиома зрительного пути», зарегистрированные в НМИЦ  нейрохирургии им. академика Н.Н. Бурденко в период с 01.01.2003 по 31.12.2015. Больные были разделены на две группы: без ДК (204 ребенка) и с наличием ДК (60 детей).

Результаты

Результаты. Нейрофиброматоз i типа (НФi) достоверно чаще встречался у детей без ДК, а детей с пиломиксоидным гистологическим вариантом было достоверно больше в группе с ДК. Пятилетняя ОВ и БСВ оказались достоверно ниже в группе детей с ДК – 82 ± 5 % и 96 ± 1 % и 37 ± 7 % и 62 ± 3 % соответственно. Выяснилось, что у девочек, пациентов без НФi, пациентов без гистологической верификации и с  пилоидными астроцитомами в группе с ДК достоверно ниже как ОВ, так и БСВ. Также оказалось, что у детей до 1 года в группе с ДК достоверно ниже ОВ, а у старших детей (более 12 мес) в группе с ДК достоверно ниже БСВ. Количество пациентов без событий оказалось достоверно выше в группе без ДК (р=0,001). Количество летальных исходов в послеоперационном периоде оказалось достоверно выше у детей с ДК (р<0,001).  Несахарный диабет и гипонатриемия достоверно чаще встречались у пациентов с диэнцефальной кахексией, а улучшение зрения после лечения достоверно чаще зафиксировано у пациентов без ДК.

Выводы

Выводы. ГЗП у пациентов с ДК имеют более агрессивное клиническое течение, что требует более внимательного лечения и последующего наблюдения.

Diencephalic cachexia (DC ) is a metabolic disorder characterized by a decrease in body weight. DC usually occurs in the presence of glioma brain tumors extended into the optic pathway. These tumors are very aggressive and have poor prognosis.

Objective

Objective: to analyze the clinical course of optic pathway gliomas (OPG s) in patients with and without DC .

Material and Methods

Material and Methods. The study included 264 patients aged 0 to 18 years with an initial diagnosis of OPG s registered in the N.N. Burdenko National Medical Research Center of neurosurgery from 01/01/2003 to 12/31/2015. Patients were divided into two groups: without DC (204 people) and with DC (60 children).

Results

Results: neurofibromatosis type I (NFI) was much more common in children without DC , and pilomyxoid histology was much more prevalent in children with DC . Five-year overall survival (OS ) and event-free survival EFS were significantly lower in children with DC than in children without DC (82 ± 5 % and 96 ± 1 %, respectively versus 37 ± 7 % and 62 ± 3 %, respectively). It was found that in the DC group, the OS and EFS rates were significantly lower in girls, in children without NFI, in children without histological verification and in children with pilocytic astrocytomas. It was also found that in the DC group, OS rates were significantly lower in children under 1 year, and EFS rates were significantly lower in children aged more than 12 months. The number of patients without events were significantly higher in the group without DC (p=0.001). The number of deaths in the postoperative period was significantly higher in children with DC (p<0.001). Diabetes insipidus and hyponatremia were significantly more common in patients with diencephalic cachexia, and vision improvement after treatment was significantly more likely to occur in patients without DC .

Conclusion

Conclusion. OPG s in patients with DC have a more aggressive clinical course, which requires more careful treatment and observation. 

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