PEDIATRIC OPTIC PATHWAY GLIOMA: DIENCEPHALIC SYNDROME
https://doi.org/10.21294/1814-4861-2021-20-1-34-45
Abstract
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: to analyze the clinical course of optic pathway gliomas (OPG s) in patients with and without DC .
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: 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. OPG s in patients with DC have a more aggressive clinical course, which requires more careful treatment and observation.
About the Authors
A. F. ValiakhmetovaRussian Federation
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
N. A. Mazerkina
Russian Federation
MD, DSc, Senior Researcher
16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia
L. I. Papusha
Russian Federation
MD, PhD, Senior Physician, Head of the Department of Optimization of CNS Tumor Therapy
1, Samory Machela Street, 117997, Moscow, Russia
O. I. Bydanov
Russian Federation
Researcher
1, Samory Machela Street, 117997, Moscow, Russia2
E. M. Tarasova
Russian Federation
MD, Pediatric Oncologist, Department of Hematology and Chemotherapy № 2
117, Leninsky pr., 119571, Moscow, Russia3
O. A. Medvedeva
Russian Federation
MD, PhD, Neurosurgeon, Children Neurosurgical Department, Junior Researcher
16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia
N. K. Serova
Russian Federation
MD, DSc, Professor, Head of the Neuroophthalmology Group, Chief Researcher
16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia
L. A. Lazareva
Russian Federation
MD, Ophthalmologist
16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia
L. V. Shishkina
Russian Federation
MD, PhD, Pathologist, Leading Specialist
16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia
Yu. Yu. Trunin
Russian Federation
MD, PhD, Radiologist, Senior Researcher
16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia
G. A. Novichkova
Russian Federation
MD, DSc, Professor, General Director
1, Samory Machela Street, 117997, Moscow, Russia
1, Ostrovityanova Street, 117997, Moscow, Russia
S. K. Gorelyshev
Russian Federation
MD, DSc, Professor, Head of the 1st Children Neurosurgical Department
16, 4th Tverskoy-Yamskaya lane, 125047, Moscow, Russia
1, Ostrovityanova Street, 117997, Moscow, Russia
A. I. Karachunsky
Russian Federation
MD, DSc, Professor, Deputy General Director
1, Samory Machela Street, 117997, Moscow, Russia
1, Ostrovityanova Street, 117997, Moscow, Russia
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Review
For citations:
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 Yu.Yu., Novichkova G.A., Gorelyshev S.K., Karachunsky A.I. PEDIATRIC OPTIC PATHWAY GLIOMA: DIENCEPHALIC SYNDROME. Siberian journal of oncology. 2021;20(1):34-45. (In Russ.) https://doi.org/10.21294/1814-4861-2021-20-1-34-45