Objective: Although bilateral vestibular schwannomas (VSs) in individual NF2 patients have the same NF2 gene mutation, they often show different growth patterns. We attempted to identify factors associated with this growth pattern inconsistency.
Patients And Methods: Cranial MR images of 120 untreated VSs in 60 NF2 patients were carefully reviewed for their growth rates. Growth pattern analysis was performed on 68 VSs in 34 NF2 patients followed up for more than three years with at least three cranial MR scans.
Results: Patient age and tumor volume were significantly associated with NF2 VS absolute growth rates (p < 0.05). Bilateral VS growth patterns in individual NF2 patients were the same in 18 (52.9 %) and different in 16 (47.1 %) patients. Patients with consistent bilateral growth patterns were significantly younger than the inconsistent patients (21.8 ± 5.8 years vs. 30.8 ± 13.1 years, p = 0.014). The bilateral VS volume consistency rates were significantly higher in patients with consistent growth patterns than in patients with inconsistent growth patterns (10/18 vs. 3/16, p = 0.028).
Conclusions: Patient age and volume consistency are the clinical risk factors for bilateral NF2 VS growth pattern inconsistencies. Bilateral VSs in young NF2 patients tend to have the same growth patterns. These findings may help us to predict the future clinical behavior of small NF2 VSs based on the past clinical history of the large ones.
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http://dx.doi.org/10.1016/j.clineuro.2020.106365 | DOI Listing |
Neuroradiol J
January 2025
Department of Neuroradiology, Mayo Clinic, USA.
Despite their similar nomenclature, Neurofibromatosis type 1 (NF1) and "Neurofibromatosis type 2" are discrete and clinically distinguishable entities. The name of "neurofibromatosis type 2" has been changed to NF2-related schwannomatosis, to reflect the fact that neurofibromas do not occur in this syndrome and therefore the name "Neurofibromatosis" is factually incorrect. Furthermore, multiple schwannomas, a hallmark feature of NF2, can also occur in patients with mutations in genes including SMARCB1 and LZTR1, all exhibiting overlapping clinical features.
View Article and Find Full Text PDFJ Neurooncol
January 2025
Department of Neurosurgery, University Hospital Leipzig, Leipzig University, Liebigstraße, 20, 04103, Leipzig, Germany.
Background: Pediatric meningiomas (PMs) are rare central nervous system tumors, accounting for 1-5% of all meningiomas, and differ from adult meningiomas in clinical, histopathological, and molecular features. Current guidelines primarily focus on adults, leaving a gap in evidence-based management for PMs. This study presents the largest meta-analysis of longitudinal individual patient data (IPD) to date, addressing progression-free survival (PFS) and overall survival (OS) in pediatric patients.
View Article and Find Full Text PDFBackground: -related schwannomatosis ( -SWN) is a debilitating condition that calls for robust treatment options. The defining feature of -SWN is the presence of bilateral vestibular schwannomas (VSs), which grow over time and can result in irreversible sensorineural hearing loss, significantly affecting the quality of life for those affected. At present, there are no FDA-approved medications specifically for treating VS or related hearing loss.
View Article and Find Full Text PDFOrphanet J Rare Dis
January 2025
Department of Neurosurgery, Helios Klinikum Erfurt, Erfurt, Germany.
Background: NF2-related schwannomatosis (NF2) is associated with various tumors of the central and peripheral nervous system. There is a wide range of disabilities these patients may suffer from and there is no validated clinical classification for disease severity. We propose a clinical classification consisting of three severity grades to assist in patient management.
View Article and Find Full Text PDFZhonghua Bing Li Xue Za Zhi
January 2025
Department of Pathology, School of Basic Medical Sciences, Peking University Third Hospital, Peking University Health Science Center, Beijing100191, China.
To understand the clinicopathological and molecular genetic characteristics of aggressive renal mucinous tubular and spindle cell carcinoma (MTSCC). The clinical features, histology, immunophenotype, molecular characteristics and prognosis of 4 cases of metastatic/recurrent renal MTSCC that were submitted to the Peking University Third Hospital (2 cases), Institute of Urology, Peking University (one case) and Zhejiang Provincial People's Hospital (one case) from 2015 to 2020 were retrospectively reviewed and analyzed. Among the four patients, two were male and two were female.
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