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Objective The extent of resection of pediatric low-grade glioma mostly improves progression-free survival. In chiasmatic hypothalamic glioma (CHG), complete resections are limited due to the relevantly high risk of associated neurological and endocrinological deficits. Still, surgery might have its role in the framework of a multidisciplinary team (MDT) approach. We report our retrospective experience from two centers on surgical options and their impact on long-term outcomes. Methods Medical records of surgically treated pediatric CHG patients between 2004 and 2022 were analyzed. Patient characteristics, surgical interventions, histology, and non-surgical therapy were retrieved together with outcome measures such as visual acuity, endocrine function, and survival. Results A total of 63 patients (33 female, NF-1, n = 8) were included. Age at first diagnosis was 4.6 years (range 0.2–16.9) and cohort follow-up was 108 ± 72 months. Twenty patients were surgically treated with a biopsy and 43 patients with debulking at a median age of 6.5 years (range 0.16–16.9). Patients received a median of 2 tumor surgeries (range 1–5). Cyst drainage was accomplished in 15 patients, and 27 patients had ventriculoperitoneal shunt implantation. Non-surgical therapy was given in 69.8%. At the end of follow-up, 74.6% of patients had stable disease. The cohort had a median Karnofsky score of 90 (range 0–100). Four patients died. Hormone substitution was necessary in 30.2%, and visual acuity was impaired in 66% of patients. Conclusion Pediatric CHG is a chronic disease due to overall high survival with multiple progressions. Surgical therapy remains a key treatment option offering biopsy, limited tumor-debulking, cyst fenestration, and hydrocephalus management in the framework of MDT decision-making. Team experience contributes to reducing possible deficits in this challenging cohort.
Objective The extent of resection of pediatric low-grade glioma mostly improves progression-free survival. In chiasmatic hypothalamic glioma (CHG), complete resections are limited due to the relevantly high risk of associated neurological and endocrinological deficits. Still, surgery might have its role in the framework of a multidisciplinary team (MDT) approach. We report our retrospective experience from two centers on surgical options and their impact on long-term outcomes. Methods Medical records of surgically treated pediatric CHG patients between 2004 and 2022 were analyzed. Patient characteristics, surgical interventions, histology, and non-surgical therapy were retrieved together with outcome measures such as visual acuity, endocrine function, and survival. Results A total of 63 patients (33 female, NF-1, n = 8) were included. Age at first diagnosis was 4.6 years (range 0.2–16.9) and cohort follow-up was 108 ± 72 months. Twenty patients were surgically treated with a biopsy and 43 patients with debulking at a median age of 6.5 years (range 0.16–16.9). Patients received a median of 2 tumor surgeries (range 1–5). Cyst drainage was accomplished in 15 patients, and 27 patients had ventriculoperitoneal shunt implantation. Non-surgical therapy was given in 69.8%. At the end of follow-up, 74.6% of patients had stable disease. The cohort had a median Karnofsky score of 90 (range 0–100). Four patients died. Hormone substitution was necessary in 30.2%, and visual acuity was impaired in 66% of patients. Conclusion Pediatric CHG is a chronic disease due to overall high survival with multiple progressions. Surgical therapy remains a key treatment option offering biopsy, limited tumor-debulking, cyst fenestration, and hydrocephalus management in the framework of MDT decision-making. Team experience contributes to reducing possible deficits in this challenging cohort.
Objective Extent of resection of pediatric low-grade glioma mostly improves progression free survival. In chiasmatic hypothalamic glioma (CHG) complete resections are limited due to high risk of associated neurological and endocrinological deficits. Still, surgery has its role in the framework of a multidisciplinary team (MDT) approach. We report our retrospective experience from two centers on surgical options and their impact on long-term outcome. Methods Medical records of surgically treated pediatric CHG patients between 2004 to 2022 were analyzed. Patient characteristics, surgical interventions, histology, and non-surgical therapy were retrieved together with outcome measures such as visual acuity, endocrine function, and survival. Results A total of 63 patients (33 female, NF-1: n = 8) were included. Age at first diagnosis was 4.6 years (range: 0.2–16.9) and cohort follow-up was 108 ± 71 months. 20 patients were surgically treated with a biopsy and 43 patients with debulking at a median age of 6.5 years (range: 0.16–16.9). Patients received a median of 2 tumor surgeries (range: 1–5). Cyst drainage was accomplished in 15 patients and 27 patients had ventriculoperitoneal shunt implantation. Non-surgical therapy was given in 69.8%. At the end of follow up 74.6% of patients had stable disease. The cohort had a median Karnofsky score of 90 (range: 0-100). Four patients died. Hormone substitution was necessary in 30.2% and visual acuity was impaired in 66% of patients. Conclusion Pediatric CHG is a chronic disease due to overall high survival with multiple progressions. Surgical therapy remains a key treatment option offering biopsy, tumor-debulking, cyst fenestration and hydrocephalus management in the framework of MDT decision making. Team experience contributes to reduce possible deficits in this challenging cohort.
Background: Brain tumors are the most common solid tumors in childhood. With the help of increased clinical awareness, improvements in imaging methods, and the identification of molecular characteristics, advances have been made in both diagnosis and treatment. Objectives: In our study, we aimed to discuss the childhood brain tumor cases followed in our clinic, along with their clinical and epidemiological features and treatment outcomes. Methods: The study group comprised children younger than 18 years old who were followed in the Pediatric Hematology and Oncology Clinic of Istanbul Medical Faculty and diagnosed with a brain tumor over the past 5 years (2018 - 2023). Symptoms, age at diagnosis, treatment options (surgery, radiotherapy, chemotherapy), and side effects were reviewed retrospectively. Results: Thirty children were diagnosed with brain tumors. Of the patients, 14 (47%) were male and 16 (53%) were female. The mean age of the children was 8.8 ± 3.1 years, and the mean age at diagnosis was 65.6 ± 37.7 months. The most common symptom was vomiting (40%). The mean duration between the onset of symptoms and diagnosis was 3.8 months, with a median of 1 month. Of the patients, 60% were diagnosed with glial tumors and 40% with non-glial tumors. Gross total resection (GTR) was performed in 26% of patients, subtotal resection (STR) in 37%, and 37% of patients did not undergo surgery. Seventy-seven percent of the patients received chemotherapy, and 57% received radiotherapy. Of the patients, 57% were considered responsive to treatment (complete/partial), 30% had stable disease, and 13% had progressive disease. The overall brain tumor mortality rate in our clinic was 13%. While 73% of our 26 patients had no or mild neurological deficits, 27% had moderate to severe disabilities (gait impairment, hearing impairment, visual impairment). Conclusion: Although the sample size was limited, it may be speculated that the surgery rate was low and long-term side effects were notable. Insufficient surgical resection can affect the treatment response and increase the risk of neurological sequelae and functional impairment. Molecular profiling should be expanded, and a palliative care team should be involved as early as possible to address long-term side effects and improve the quality of life for patients.
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