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Pediatric vestibular schwannoma without evidence of neurofibromatosis: consecutive 18 microsurgical experiences

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Abstract

Purpose

Sporadic vestibular schwannoma (VS) is rare in children in contrast to adults, and detailed investigations of case series of these patients using a single fixed protocol are scarce. This study presents our surgical experience of pediatric VSs without clinical evidence of neurofibromatosis type 2 (NF2) at the initial diagnosis.

Methods

Among 1385 consecutive sporadic VS surgeries, 18 pediatric patients (1.3%; 11–18 years old) were retrospectively investigated.

Results

The most common initial symptom was hearing disturbance (72.2%), and 6 patients (33.3%) experienced a delayed diagnosis (over 2 years after initial symptom onset). Preoperative image characteristics of these tumors included a solid tumor, hypervascularity, and significant extension into the internal acoustic meatus, when compared with adults. Preoperative embolization was successfully accomplished for 2 recent hypervascular tumors. The tumor resection rate was 95–100% under sufficient intraoperative neuromonitoring, and no additional surgery was required during the follow-up period (average: 57.9 months). No patients experienced permanent facial nerve palsy, and serviceable hearing function was preserved in 6 of 11 patients. Signs of NF2, such as bilateral VSs, were not identified in any patients during the follow-up.

Conclusion

Safe and sufficient tumor resection was achieved under detailed neuromonitoring in pediatric patients with sporadic VS, although this tends to be difficult owing to hypervascularity, a small cranium, and significant meatal extension. Preoperative embolization may help safe resection of hypervascular tumors. Subsequent development of NF2 has not been observed up to the most recent follow-up, but careful observation is essential for these younger patients.

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Abbreviations

ASL:

Arterial spin labeling

ABR:

Auditory brainstem response

CNAP:

Cochlear nerve action potentials

CT:

Computed tomography

MRI:

Magnetic resonance imaging

NBCA:

N-Butyl cyanoacrylate

NF2:

Neurofibromatosis type 2

QOL:

Quality of life

VS:

Vestibular schwannoma

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Acknowledgements

We thank Ms. Eriko Hikawa and Ms. Yuri Miyazaki for their assistance in preparing this manuscript.

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Authors and Affiliations

  1. Department of Neurosurgery, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan

    Ken Matsushima, Michihiro Kohno, Norio Ichimasu & Nobuyuki Nakajima

  2. Department of Neurosurgery, Tokyo Metropolitan Police Hospital, Tokyo, Japan

    Michihiro Kohno & Masanori Yoshino

Authors
  1. Ken Matsushima
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  2. Michihiro Kohno
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  3. Norio Ichimasu
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  4. Nobuyuki Nakajima
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  5. Masanori Yoshino
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Corresponding author

Correspondence to Michihiro Kohno.

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Ethics approval

This study was approved by the ethics committees of Tokyo Medical University (study approval no.: TS2020-0365) and Tokyo Metropolitan Police Hospital (study approval no.: 20-A28), Japan.

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The patients and their parents gave consent to use their information and images for research purposes.

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The patients and their parents gave consent regarding the publication of their information and images for publication.

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The authors declare no competing interests in association with this paper.

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Matsushima, K., Kohno, M., Ichimasu, N. et al. Pediatric vestibular schwannoma without evidence of neurofibromatosis: consecutive 18 microsurgical experiences. Childs Nerv Syst 38, 1505–1512 (2022). https://doi.org/10.1007/s00381-022-05477-9

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  • DOI: https://doi.org/10.1007/s00381-022-05477-9

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