Sporadic, unilateral pediatric-spectrum vestibular schwannomas (PSVSs), which occur in patients 21 years or younger without a diagnosis of neurofibromatosis type 2, are rare tumors with an estimated incidence of less than 0.1/100,000 children. A subset of PSVS tumors (designated Koos grade IV PSVSs) exhibits significant brainstem compression and poses unique management challenges.

Vestibular schwannomas in paediatric patients without neurofibromatosis type 2 are rare; this single case adds to the limited literature but does not change standard audiological monitoring or referral protocols.

Sporadic vestibular schwannomas in the paediatric population are exceptionally rare, and documenting surgical outcomes in this group is important for informing future management guidelines.

1. 01Case report of a Koos grade IV (large, involving the brainstem) vestibular schwannoma in a paediatric patient.
2. 02Tumour was sporadic — patient did not have neurofibromatosis type 2, the usual genetic cause in young patients.
3. 03Gross-total resection (complete surgical removal) was achieved.
4. 04Published in Surgical Neurology International; PMID 42116902.
5. 05Literature review component summarises prior paediatric sporadic vestibular schwannoma cases.

PMID

42116902

DOI

10.25259/SNI_1446_2025.

Journal

Surgical Neurology International

Publication type

case_report

Evidence level

4

Sample size

1

Population

Paediatric patient with sporadic Koos grade IV vestibular schwannoma without neurofibromatosis type 2

Intervention

Gross-total surgical resection