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Volume 12
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Article

Age at Onset and Presenting Symptoms of Neurofibromatosis Type 2 as Prognostic Factors for Clinical Course of Vestibular Schwannomas

by
Isabel Gugel
1,2,*,
Florian Grimm
1,
Julian Zipfel
1,2,3,
Christian Teuber
1,
Ulrike Ernemann
4,
Lan Kluwe
5,6,
Marcos Tatagiba
1,2,
Victor-Felix Mautner
2,5 and
Martin Ulrich Schuhmann
1,2,3
1
Department of Neurosurgery, University Hospital Tübingen, 72076 Tübingen, Germany
2
Centre of Neurofibromatosis and Rare Diseases, University Hospital Tübingen, 72076 Tübingen, Germany
3
Division of Pediatric Neurosurgery, University Hospital Tübingen, 72076 Tübingen, Germany
4
Department of Neuroradiology, University Hospital Tübingen, 72076 Tübingen, Germany
5
Department of Neurology, University Medical Center Hamburg-Eppendorf, 20251 Hamburg, Germany
6
Department of Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, 20251 Hamburg, Germany
*
Author to whom correspondence should be addressed.
Cancers 2020, 12(9), 2355; https://doi.org/10.3390/cancers12092355
Submission received: 14 June 2020 / Revised: 17 August 2020 / Accepted: 18 August 2020 / Published: 20 August 2020
(This article belongs to the Special Issue Schwannomas – So-Called Benign Nervous System Tumors)
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Abstract

The presenting symptoms of the tumor suppressor gene syndrome neurofibromatosis type 2 (NF2) are often non-specific and unrelated to the disease hallmark bilateral vestibular schwannomas (VS). However, age at onset and presenting symptoms may have predictive values for the clinical course of VS. In this retrospective single-center study, we addressed this issue by reviewing 106 patients with 194 VS. Presenting symptoms attributable to VS commonly occur in 87% of adults and 31% of children. Age at onset significantly correlates with tumor volumes at presentation (p = 0.034). In addition, age at onset significantly correlates with pure-tone average (p = 0.0001), speech discrimination scores (p = 0.001), age at beginning of hearing loss (p = 0.0001), age at deafness (p = 0.0001), and age at first surgery (p = 0.0001). Patients presenting with VS related symptoms had significantly (p < 0.05) worse hearing values at presentation and after surgery. These patients also exhibited higher growth rates and tumor volumes compared to patients with non-VS related presenting symptoms, but this difference did not reach the significance level of p < 0.05. Due to the late appearance of these symptoms, the time of beginning hearing loss, surgery and deafness is significantly delayed (p < 0.05) compared to patients not presenting with VS. In summary, age at onset and type of presenting symptom provide excellent prognostic parameters for predicting VS- and hearing-related clinical course.
Keywords: presenting symptom; hearing preservation; neurofibromatosis type 2; growth rate; vestibular schwannoma presenting symptom; hearing preservation; neurofibromatosis type 2; growth rate; vestibular schwannoma

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MDPI and ACS Style

Gugel, I.; Grimm, F.; Zipfel, J.; Teuber, C.; Ernemann, U.; Kluwe, L.; Tatagiba, M.; Mautner, V.-F.; Schuhmann, M.U. Age at Onset and Presenting Symptoms of Neurofibromatosis Type 2 as Prognostic Factors for Clinical Course of Vestibular Schwannomas. Cancers 2020, 12, 2355. https://doi.org/10.3390/cancers12092355

AMA Style

Gugel I, Grimm F, Zipfel J, Teuber C, Ernemann U, Kluwe L, Tatagiba M, Mautner V-F, Schuhmann MU. Age at Onset and Presenting Symptoms of Neurofibromatosis Type 2 as Prognostic Factors for Clinical Course of Vestibular Schwannomas. Cancers. 2020; 12(9):2355. https://doi.org/10.3390/cancers12092355

Chicago/Turabian Style

Gugel, Isabel, Florian Grimm, Julian Zipfel, Christian Teuber, Ulrike Ernemann, Lan Kluwe, Marcos Tatagiba, Victor-Felix Mautner, and Martin Ulrich Schuhmann. 2020. "Age at Onset and Presenting Symptoms of Neurofibromatosis Type 2 as Prognostic Factors for Clinical Course of Vestibular Schwannomas" Cancers 12, no. 9: 2355. https://doi.org/10.3390/cancers12092355

APA Style

Gugel, I., Grimm, F., Zipfel, J., Teuber, C., Ernemann, U., Kluwe, L., Tatagiba, M., Mautner, V.-F., & Schuhmann, M. U. (2020). Age at Onset and Presenting Symptoms of Neurofibromatosis Type 2 as Prognostic Factors for Clinical Course of Vestibular Schwannomas. Cancers, 12(9), 2355. https://doi.org/10.3390/cancers12092355

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