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OBJECTIVE: To identify factors for tumor relapse and poor outcome in patients with meningiomas in the first two decades of life. METHODS: All patients ≤â¯21 years of age who underwent resection of a meningioma at the department of neurosurgery, Medical University of Vienna between 1989 and 2022 were included in this retrospective study. Clinical and radiological data were extracted from the medical records. Outcome and tumor relapse were analyzed for tumor location, histological findings and extent of resection. RESULTS: In this study 18 patients were included, 6 meningiomas were located in the skull base, 5 in the convexity and 7 in other locations including intraventricular and spine (2 patients each), falx, intraparenchymal and optic nerve sheath. Most frequent symptoms were seizures and cranial nerve palsy. In total 56% of the meningiomas were World Health organization (WHO) grade 1, 39% grade 2 and 5% grade 3. Gross total resection was achieved in 67%. The overall relapse rate was 61% and 50% underwent repeat surgery. All patients with convexity meningiomas became seizure free and had a favorable outcome. Relapse and clinical outcome were independent of WHO grade among the whole cohort but the outcome significantly depended on the WHO grade when patients with skull base meningiomas were analyzed as a subgroup. The relapse rate was significantly higher in cases of skull base location (100% vs. 42%, pâ¯= 0.038) and after subtotal resection (100% vs. 42%, pâ¯= 0.038). Clinical outcome was also significantly worse and the rate of complications was higher in patients with skull base meningiomas. CONCLUSION: Patients with convexity meningiomas in the first two decades of life have a good outcome due to high chance of gross total resection. Patients with skull base meningioma are at high risk of relapse and poor outcome, particularly those with WHO grades 2 and 3. Subtotal resection in patients with skull base location is probably the main reason for this difference.
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BACKGROUND: Tumors of the fourth ventricle are frequently treated pathologies in pediatric neurosurgery. Data regarding predictors for permanent neurological deficits, long-term functional outcomes, cerebellar mutism (CM), the extent of resection (EOR), and oncological outcomes are scarce. We attempt to contribute to this topic with an analysis of our institutional cohort. METHODS: A retrospective single-center study of patients aged ≤ 19 years who underwent primary surgical resection of a fourth ventricular tumor over a 15-year period (2006-2021). Predictors analyzed included age, gender, surgical approach, anatomical pattern, tumor grade, EOR, tumor volume, and others as appropriate. RESULTS: One hundred six patients were included (64 males, mean age 7.3 years). The rate of permanent neurological deficit was 24.2%; lateral tumor extension (p = 0.036) and tumor volume greater than 38 cm3 (p = 0.020) were significant predictors. The presence of a deficit was the only significant predictor of reduced (less than 90) Lansky score (p = 0.005). CM occurred in 20.8% of patients and was influenced by medulloblastoma histology (p = 0.011), lateral tumor extension (p = 0.017), and male gender (p = 0.021). No significant difference between the transvermian and telovelar approach in the development of CM was detected (p = 0.478). No significant predictor was found for the EOR. EOR was not found to be a significant predictor of overall survival for both low-grade and high-grade tumors; however, gross total resection (GTR) was protective against tumor recurrence compared to near-total or subtotal resection (p < 0.001). In addition, survival was found to be better in older patients (≥ 7.0 years, p = 0.019). CONCLUSION: The overall rate of postoperative complications remains high due to the eloquent localization. Older patients (> 7 years) have been found to have better outcomes and prognosis. Achieving GTR whenever feasible and safe has been shown to be critical for tumor recurrence. CM was more common in patients with medulloblastoma and in patients with tumors extending through the foramen of Luschka. The telovelar approach uses a safe and anatomically sparing corridor; however, it has not been associated with a lower incidence of CM and neurological sequelae in our series, showing that each case should be assessed on an individual basis.
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Neoplasias Cerebelares , Meduloblastoma , Humanos , Criança , Masculino , Idoso , Quarto Ventrículo/diagnóstico por imagem , Quarto Ventrículo/cirurgia , Estudos Retrospectivos , Procedimentos Neurocirúrgicos/efeitos adversos , Meduloblastoma/cirurgia , Recidiva Local de Neoplasia/cirurgia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Neoplasias Cerebelares/cirurgia , Neoplasias Cerebelares/etiologia , Resultado do TratamentoRESUMO
PURPOSE: Current management of pediatric intramedullary ependymoma is extrapolated from adult series since large studies in children are unavailable. This has led us to share our experience with this rare tumor and compare it to the literature and to review and highlight important aspects of current management and point out inconsistencies. METHODS: This is a retrospective analysis of patients with intramedullary ependymoma managed at our institution between 2004 and 2021. RESULTS: During the study period, 5 patients were treated for intramedullary ependymoma. Cases of myxopapillary ependymoma were excluded. The mean age of our cohort was 11.2 years. We identified 4 cases of grade II ependymoma and 1 case of grade III ependymoma. Gross tumor removal (GTR) was achieved in two patients (40%) of patients. One patient was treated with radiotherapy for recurrence and two patients received chemotherapy. There were no cases of recurrence among patients treated with GTR, but in all patients treated with STR. Eighty percent of patients either improved or stayed stable neurologically. During follow-up (mean 73 months), 2 patients died of disease. CONCLUSION: GTR and tumor grade remain the key prognostic factor of long-term tumor-free survival. Many questions prevail regarding outcomes, correct use of adjuvant therapy, and prognostic factors.
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Ependimoma , Neoplasias da Medula Espinal , Adulto , Humanos , Criança , Estudos Retrospectivos , Procedimentos Neurocirúrgicos , Terapia Combinada , Ependimoma/cirurgia , Ependimoma/patologia , Neoplasias da Medula Espinal/cirurgia , Neoplasias da Medula Espinal/patologiaRESUMO
PURPOSE: Literature dedicated to growth patterns and growth rate influencing factors of radiation-induced meningiomas (RIMs) is limited. To deliver new insights into the topic, a volumetric growth analysis of RIMs was performed. METHODS: This single-center, retrospective cohort study included patients diagnosed with intracranial meningioma who received radiation treatment at least > 5 years before the RIM diagnosis. Volumetric analysis of individual RIMs was performed using 3D volumetry at the time of RIM diagnosis and during follow-up. RIM growth was determined by calculating absolute (AGR), and relative (RGR) growth rates. Prognostic factors associated with RIM growth were evaluated. RESULTS: A total of 26 patients with 33 meningiomas were enrolled in the study and radiologically/clinically followed up during a median duration of 5.6 years (IQR 3.9-8.8 years). Median AGR was 0.19 cm3 per year and the median RGR was 34.5% per year. Surgically managed RIMs were more likely fast-growing compared to observed ones based on the AGR (p < 0.002). The recurrence rate after total resection was 14.3%. Younger age at RIM diagnosis was associated with higher tumor growth (RGR ≥ 30%, p = 0.040). A significant correlation was found between the length of latency period and the RGR (p = 0.005). CONCLUSION: To diagnose RIM as early as possible comprehensive MRI surveillance is required. Younger patients with shorter latency periods may profit from shortened MRI intervals, with further management being dependent on the growth rate and eventual symptomatology.