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.
✦ The floor
Discussion
Signed responses from readers of the wire.
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.
- 01Case report of a Koos grade IV (large, involving the brainstem) vestibular schwannoma in a paediatric patient.
- 02Tumour was sporadic — patient did not have neurofibromatosis type 2, the usual genetic cause in young patients.
- 03Gross-total resection (complete surgical removal) was achieved.
- 04Published in Surgical Neurology International; PMID 42116902.
- 05Literature review component summarises prior paediatric sporadic vestibular schwannoma cases.
Gross-total resection of a Koos grade IV sporadic vestibular schwannoma is surgically achievable in a paediatric patient without NF2.
studypartially supported- 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
Primary outcomes
Extent of tumour resection; Surgical outcome and complications