Vestibular schwannoma growth in patients with neurofibromatosis Type 2: a longitudinal study

Mautner, Victor‐Felix; Baser, Michael E.; Thakkar, S. D.; Feigen, U.; Friedman, Jan M.; Kluwe, Lan

doi:10.3171/jns.2002.96.2.0223

Cited by 98 publications

(64 citation statements)

References 31 publications

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“…Several single-institution clinical series have been reported from NF2 centers, but these have typically been small and retrospective. [7][8][9][10][11] In addition, many of these studies have reported results primarily as the average growth rates or hearing decline among all subjects. This measure is less useful in designing clinical trials for drug treatment of NF2-related VS. For this purpose, an understanding of individual times to failure for measures such as tumor growth or hearing decline in untreated patients is required.…”

Section: Introductionmentioning

confidence: 99%

Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Plotkin¹,

Merker²,

Muzikansky³

et al. 2014

Otology &Amp; Neurotology

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Section: Introductionmentioning

confidence: 99%

Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Plotkin¹,

Merker²,

Muzikansky³

et al. 2014

Otology &Amp; Neurotology

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“…Unbehandelt führen vestibuläre Schwannome zur Hirnstammkompression und Ertaubung. Das Wachstum dieser Tumoren variiert stark, wobei eine inverse Korrelation zum Alter zu beobachten ist [16,33].…”

Section: Vestibuläre Schwannomeunclassified

Neurofibromatose Typ 2

Mautner

2009

Medizinische Genetik

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Zusammenfassung Neurofibromatose Typ 2 (NF2) ist eine genetische Erkrankung, die autosomal-dominant vererbt wird und durch multiple Tumoren des Nervensystems gekennzeichnet ist. Bilaterale vestibuläre Schwannome, bekannt als Akustikusneurinome, sind das Hauptmerkmal der Erkrankung und treten bei etwa 90% der Betroffenen auf. Sie beeinträchtigen das Hörvermögen der Patienten und führen nicht selten zur Ertaubung. Ebenso häufig (>90%) treten bei NF2 spinale Tumoren (Schwannome, Meningiome und Ependymome) auf, die in etwa 1/3 der Fälle zu neurologischen Defiziten führen. Daneben finden sich zerebrale Schwannome und Meningiome als typische NF2-assoziierte Tumormanifestationen. Ferner kennzeichnen Polyneuropathie und okuläre Abnormalitäten das Krankheitsbild. Die genetische Ursache der NF2 ist eine heterozygote Inaktivierung des NF2-Tumorsuppressorgens, welches auf Chromosom 22q lokalisiert ist. Die Inzidenz der NF2 beträgt etwa 1:25.000 Geburten, wobei 50–80% der Patienten keine familiäre Belastung aufweisen. Bei Patienten mit Neumutationen (de novo) wurde eine Mosaikbildung mit überraschend hoher Frequenz (25–30%) nachgewiesen. Der vorliegende Beitrag beschreibt Klinik und Genetik der NF2 sowie aktuelle Entwicklungen in der medikamentösen Therapie.

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“…1,5,10,18,21 Our study showed 0.33 cm 3 /year of actuarial growth rate, suggesting a slow natural course of VS in children. Our study suggests that the natural growth rate may be slow only in children.…”

Section: Vestibular Schwannoma In Children With Nf2mentioning

confidence: 99%

“…9 The mean growth rate of VS was estimated using simple linear regression of the VS volume over the full follow-up period before any treatment as previously described. 5,9,21,39 …”

Section: Image Analysis For Measuring the Natural Growth Rate Of Vsmentioning

confidence: 99%

Clinical course of vestibular schwannoma in pediatric neurofibromatosis Type 2

Choi

Lee

Wang

et al. 2014

PED

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Object Neurofibromatosis Type 2 (NF2) is an autosomal-dominant inherited disease, characterized by multiple neoplasia syndromes, including meningioma, schwannoma, glioma, and ependymoma. In this report, the authors present their clinical experience with pediatric NF2 patients. In particular, they focused on the clinical course of vestibular schwannoma (VS), including the natural growth rate, tumor control, and functional hearing outcomes. Methods From May 1988 to June 2012, the authors recruited patients who were younger than 18 years and fulfilled the Manchester criteria. In total, 25 patients were enrolled in this study. The authors analyzed the clinical course of these patients. In addition, they measured the natural growth rate of VS before any treatment in these children with NF2. Then, they evaluated the tumor control rate and functional hearing outcomes after the treatment of VS. Results The mean age at the onset of NF2-related symptoms was 9.9 ± 4.5 years (mean ± SD, range 1–17 years). The mean age at the diagnosis of NF2 was 12.9 ± 2.9 years (range 5–17 years). The mean follow-up period was 89.3 months (range 12–311 months). As initial manifestations, nonvestibular symptoms were frequently observed in pediatric patients with NF2. The mean natural growth rate of VS was 0.33 ± 0.41 cm3/year (range 0–1.35 cm3/year). The tumor control rate of VS was 35.3% at 3 years after Gamma Knife surgery (GKS). The actuarial rate of useful hearing preservation was 67% in the 1st year and 53% in the 5th year after GKS. Conclusions Clinical manifestations in children with NF2 were highly variable, compared with their adult counterparts. The natural growth rate of VS in children is slow, and this oncological feature may explain the diverse clinical manifestations besides vestibular symptoms in children with NF2. The treatment outcome of GKS for VS in children with NF2 was not favorable compared with previous reports of affected adults.

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Vestibular schwannoma growth in patients with neurofibromatosis Type 2: a longitudinal study

Abstract: There is considerable variability in growth rates of VSs in patients with NF2, but they tend to be higher in patients who are younger at onset of signs or symptoms.

Cited by 98 publications

References 31 publications

Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Neurofibromatose Typ 2

Clinical course of vestibular schwannoma in pediatric neurofibromatosis Type 2

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