Real Incidence of Vestibular Schwannoma? Estimations From a National Registry

Kleijwegt, Maarten; Ho, Vincent K.Y.; Visser, Otto; Godefroy, Willem P.; Mey, Andel van der

doi:10.1097/mao.0000000000001169

Cited by 64 publications

(74 citation statements)

References 18 publications

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“…VS is the most common cerebellopontine angle lesion, with an approximate incidence of 10 to 25 per million per year. 4,5,9 Interestingly, there has been a welldescribed upward trend in Fig. 2 Graphic illustrating the main symptom that led to diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…By contrast, retrospectively collected survey data provides an opportunity to directly address questions that are so specifically focused on the patient experience, a research methodology that is powerfully enhanced in the assessment of low-incidence diseases like VS via patient run associations or support groups that empower the collection of data from large populations in a manner not restricted to an institution, or a single surgeon's experience. [4][5][6][7] With these principles in mind, our objective was to retrospectively review a large cohort of VS patients, and capture the most common chief complaints underlying their presentation. In other words, the driving symptom that eventually leads to the VS diagnosis as well as the self-reported tumor size at diagnosis, to better understand trends in disease presentation and natural history, identify possible opportunities for earlier intervention, and correlate patient presentation characteristics with age and year of diagnosis.…”

Section: Introductionmentioning

confidence: 99%

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Main Symptom that Led to Medical Evaluation and Diagnosis of Vestibular Schwannoma and Patient-Reported Tumor Size: Cross-sectional Study in 1,304 Patients

et al. 2018

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Objectives Although vestibular schwannomas (VS) are known to cause cranial nerve deficits, cerebellar symptoms, and hydrocephalus, the role of these symptoms as the key driver of presentation from the patient's perspective has not been described. Our objective was to survey a large, retrospective VS cohort to document the patient-reported principal initial symptom, and self-reported tumor size, and to study trends in VS patient presentation. Methods Patients diagnosed with VS at our tertiary referral center and belonging to the Acoustic Neuroma Association (ANA) answered a questionnaire between 2015 and 2017. Demographic data, self-reported tumor size, and symptomatology were analyzed. Results 1,304 patients completed the questionnaire. Tumors were diagnosed from 1966 to 2017 at a mean 51.8 years (range: 8–86 years); 66% were female, and 1.1% had confirmed neurofibromatosis type 2 (NF2). Tumor size was reported using a 6-point scale: 0 to 1 cm (22.9%), 1 to 2 cm (28.7%), 2 to 3 cm (20.5%), 3 to 4 cm (10%), greater than 4 cm (7.2%), and unknown (10.6%). Hearing loss was the most common symptom that led to diagnosis (51.5%), followed by dizziness (17%), tinnitus (11.2%), and incidental diagnosis (10.2%); a fraction that has increased significantly in the last decade (p = 0.022). Larger tumors and NF2 were significantly associated with young age (p < 0.001). Conclusion Our large-scale questionnaire-driven review of 1,304 patients confirms that VS presentations are stereotypical, with most individuals recalling hearing loss, dizziness, or tinnitus as their chief complaint. Many tumors were incidentally diagnosed; an expanding population, attributable to increased access to magnetic resonance imaging (MRI). Large tumors were significantly more prevalent among younger patients at diagnosis, excluding NF2 patients, suggesting a more aggressive tumor biology that remains incompletely understood.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Main Symptom that Led to Medical Evaluation and Diagnosis of Vestibular Schwannoma and Patient-Reported Tumor Size: Cross-sectional Study in 1,304 Patients

et al. 2018

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“…These tumors make up 8% of the primary brain tumors diagnosed in the United States 1 and have an incidence of 37.5 per million inhabitants in the Netherlands. 2 In the last few decades, the main treatment goal for VS has shifted from complete removal of the tumor to functional preservation of the facial, trigeminal, and cochlear nerves. 3 Especially the introduction of less invasive treatment options and their reduced risks at post-treatment morbidities has led to this substantial shift.…”

Section: Introductionmentioning

confidence: 99%

Prediction of transient tumor enlargement using MRI tumor texture after radiosurgery on vestibular schwannoma

et al. 2020

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Purpose: Vestibular schwannomas (VSs) are uncommon benign brain tumors, generally treated using Gamma Knife radiosurgery (GKRS). However, due to the possible adverse effect of transient tumor enlargement (TTE), large VS tumors are often surgically removed instead of treated radiosurgically. Since microsurgery is highly invasive and results in a significant increased risk of complications, GKRS is generally preferred. Therefore, prediction of TTE for large VS tumors can improve overall VS treatment and enable physicians to select the most optimal treatment strategy on an individual basis. Currently, there are no clinical factors known to be predictive for TTE. In this research, we aim at predicting TTE following GKRS using texture features extracted from MRI scans. Methods: We analyzed clinical data of patients with VSs treated at our Gamma Knife center. The data was collected prospectively and included patient-and treatment-related characteristics and MRI scans obtained at day of treatment and at follow-up visits, 6, 12, 24 and 36 months after treatment. The correlations of the patient-and treatment-related characteristics to TTE were investigated using statistical tests. From the treatment scans, we extracted the following MRI image features: first-order statistics, Minkowski functionals (MFs), and three-dimensional gray-level co-occurrence matrices (GLCMs). These features were applied in a machine learning environment for classification of TTE, using support vector machines. Results: In a clinical data set, containing 61 patients presenting obvious non-TTE and 38 patients presenting obvious TTE, we determined that patient-and treatment-related characteristics do not show any correlation to TTE. Furthermore, first-order statistical MRI features and MFs did not significantly show prognostic values using support vector machine classification. However, utilizing a set of 4 GLCM features, we achieved a sensitivity of 0.82 and a specificity of 0.69, showing their prognostic value of TTE. Moreover, these results increased for larger tumor volumes obtaining a sensitivity of 0.77 and a specificity of 0.89 for tumors larger than 6 cm 3 . Conclusions: The results found in this research clearly show that MRI tumor texture provides information that can be employed for predicting TTE. This can form a basis for individual VS treatment selection, further improving overall treatment results. Particularly in patients with large VSs, where the phenomenon of TTE is most relevant and our predictive model performs best, these findings can be implemented in a clinical workflow such that for each patient, the most optimal treatment strategy can be determined.

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“…1,2 Incidence in the Netherlands is assumed to be 1.6 per 100,000 persons a year, based on several Danish studies. [3][4][5][6] The incidence has increased over the years, probably due to the wide availability of advancing magnetic resonance ima-ging (MRI) techniques, as well as higher awareness among doctors and patients.…”

Section: Introductionmentioning

confidence: 99%

Clinical Predictors Leading to Change of Initial Conservative Treatment of 836 Vestibular Schwannomas

et al. 2019

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Objective This study was aimed to determine the role of clinical presentation and tumor characteristics in vestibular schwannoma (VS) at diagnosis, initially treated with conservative management. Design The study was designed as a retrospective chart review. Setting The study was prepared at national tertiary referral center for VS patients. Participants A total of 836 VS patients, initially treated conservatively, were included. Main Outcome Measures Patient characteristics: age at diagnosis, gender, frequency, and duration of, hearing loss, tinnitus, balance disorder (unsteadiness, dizziness, and vertigo), respectively; and tumor characteristics: laterality, growth, cystic component, and location were analyzed in relation to tumor size at diagnosis and change in treatment strategy. Results In total, 169 (20%) patients had a change in treatment strategy. Factors at diagnosis that had a high influence on intervention were a short duration of hearing loss (hazard ratio [HR]: 4.8, p < 0.001) and cystic tumors (HR = 2.6, p < 0.001). Balance disorders and extracanalicular (EC) tumor location have a medium influence on intervention (HR = 1.6, p < 0.01). Tumour growth was seen in 55% of the intervention group; we found a significant correlation with a short duration of hearing loss. Cystic VS was significantly higher between the medium and large tumors, 24.3% and 38.1%. (p = 0.001), respectively. Conclusions Patients with a short duration of hearing loss, balance disorders, EC located tumors, and cystic tumors have a significantly higher chance of a change in treatment strategy. Large tumor size at diagnosis and a cystic component were related to age > 65 years at diagnosis.

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Real Incidence of Vestibular Schwannoma? Estimations From a National Registry

Abstract: Completeness of the registration of VS varies across regions in The Netherlands. Incidence estimates obtained from regions with the highest rates are higher than those reported by previous studies.

Cited by 64 publications

References 18 publications

Main Symptom that Led to Medical Evaluation and Diagnosis of Vestibular Schwannoma and Patient-Reported Tumor Size: Cross-sectional Study in 1,304 Patients

Main Symptom that Led to Medical Evaluation and Diagnosis of Vestibular Schwannoma and Patient-Reported Tumor Size: Cross-sectional Study in 1,304 Patients

Prediction of transient tumor enlargement using MRI tumor texture after radiosurgery on vestibular schwannoma

Clinical Predictors Leading to Change of Initial Conservative Treatment of 836 Vestibular Schwannomas

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