Optic pathway gliomas in children: prognostic factors, response assessment, role of carboplatin and vincristine chemotherapy regime

Валиахметова, Э. Ф.; Быданов, О. И.; Gorelyshev, Sergey; Serova, N K; Лазарева, Л. А.; Shishkina, L V; Mazerkina, Nadezhda; Трунин, Ю. Ю.; Chulkova, S. V.; Грищенко, Н. В.; Егорова, А. В.; Papusha, Ludmila; Novichkova, Galina; Карачунский, А. И.

doi:10.24287/1726-1708-2019-18-1-62-72

Cited by 5 publications

(1 citation statement)

References 25 publications

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“…Результаты эффективности данного режима в нашей стране у пациентов с глиомами зрительного пути были опубликованы Э.Ф. Валиахметовой и соавт., показатели выживаемости в данной работе сравнимы с данными зарубежных исследований [3].…”

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Low-grade gliomas with the V600E mutation in the BRAF gene in children: clinical features and treatment options

Papusha

Валиахметова

Druy

et al. 2020

Voprosy gematologii/onkologii i immunopatologii v pediatrii

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The main pathogenetic mechanism of the development of pediatric low grade gliomas (pLGGs) is genetic aberrations in BRAF gene. This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. We analyzed the clinical and molecular characteristics of 69 patients with LGGs. Molecular genetic testing for BRAF V600E mutation was performed by allele-specific real-time PCR and Sanger sequencing. BRAF V600E mutation was detected in 15 (21.7%) patients with LGG. The majority of BRAF-mutated cases of LGGs had the midline location: OPG – 7, subcortical ganglia – 1, brainstem – 2. The 2-year PFS was much worse in patients with BRAF V600E compared to patients without this mutation – 30% and 66.2%, respectively. The median time to progression for patients with BRAF V600E mutation was 9.5 months compared to 3.1 years for patients without indicated substitution. 5 patients with BRAF V600E-mutated LGGs who experienced progression after the conventional treatment, received targeted therapy (BRAF-inhibitor-3, BRAF + MEK inhibitors – 2) with good response (complete response – 2, partial response – 3). BRAF V600E mutation contributes to poor outcome in patients with LGGs Targeted therapy could be effective in this cohort of patients.

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Low-grade gliomas with the V600E mutation in the BRAF gene in children: clinical features and treatment options

Papusha

Валиахметова

Druy

et al. 2020

Voprosy gematologii/onkologii i immunopatologii v pediatrii

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Glioma of the anterior visual pathway in children. Part II. Current treatment trends

Alyaeva,

Serova

2024

Russian Pediatric Ophthalmology

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The literature review presents modern treatment aspects for anterior optic pathway gliomas (OPGs), which are low-grade brain tumors accounting for 20%–30% of childhood gliomas, can occur anywhere along the visual pathways and in the development of the surrounding structure, and are predominantly benign. The predominant histological type of tumor in this localization is piloid astrocytoma (PA), less commonly pilomyxoid astrocytoma (PMA). However, the course of anterior OPGs is unpredictable and varies from spontaneous regression to progression with severe visual, neurological, and endocrine disorders, affecting treatment and disease prognosis. Although there have been advances in clinical studies based on histological and molecular genetic analyses, no fundamental changes in survival rates and recurrence-free periods and improvements in functional outcomes have been achieved. Furthermore, no studies have comprehensively analyzed the functional results depending on the management tactics of pediatric patients with anterior visual pathway gliomas. Anterior optic pathway glioma treatment is challenging and complex problem, which depends on the patient’s age, clinical picture, localization, surgical resectability, and histological and molecular genetic study results. It includes surgical treatment, chemotherapy, radiation therapy, the use of targeted therapy drugs, and additional advanced techniques that are still under development and research. Optimal treatment of anterior optic pathway gliomas in children remains a topic of discussion in the current literature.

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Pediatric Optic Pathway Glioma: Diencephalic Syndrome

Валиахметова

Mazerkina²,

Papusha

et al. 2021

Sib. onkol. ž.

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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.

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Optic pathway gliomas in children: prognostic factors, response assessment, role of carboplatin and vincristine chemotherapy regime

Abstract: п е р с п е к т и в н ы е и с с л е д о в а н и я

Cited by 5 publications

References 25 publications

Low-grade gliomas with the V600E mutation in the BRAF gene in children: clinical features and treatment options

Low-grade gliomas with the V600E mutation in the BRAF gene in children: clinical features and treatment options

Glioma of the anterior visual pathway in children. Part II. Current treatment trends

Pediatric Optic Pathway Glioma: Diencephalic Syndrome

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