Sporadic sarcoma arising from a previously resected benign vestibular schwannoma without neurofibromatosis or radiosurgery: illustrative case

Vestibular schwannomas (VSs) are common benign tumors of the cerebellopontine angle, often treated with surgery or stereotactic radiosurgery to preserve nerve function. Malignant transformation of VS (MTVS) is exceedingly rare and has been primarily associated with neurofibromatosis type 1 (NF1), neurofibromatosis type 2-related schwannomatosis (NF2-SWN), or prior radiation exposure....

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This single illustrative case raises awareness of an extremely rare malignant transformation at a prior vestibular schwannoma resection site without known predisposing factors; no change to routine clinical practice is warranted based on a single case report.

This case highlights an exceptionally rare but serious oncological complication following vestibular schwannoma resection, potentially expanding the differential for recurrent skull base lesions.

1. 01A sporadic sarcoma arose at the site of a previously resected benign vestibular schwannoma with no neurofibromatosis or prior radiosurgery.
2. 02Case challenges the assumption that malignant transformation requires radiation exposure or genetic predisposition (NF2).
3. 03Published as an illustrative case in Journal of Neurosurgery: Case Lessons 2026 (PMID 42546347).
4. 04Clinicians should consider malignant recurrence in the differential for post-surgical skull base lesions.
5. 05Extremely rare event; no epidemiological conclusions can be drawn from a single case.

PMID

42546347

DOI

10.3171/CASE251003.

Journal

Journal of Neurosurgery: Case Lessons

Publication type

case_report

Evidence level

4

Sample size

1

Population

Single patient with prior benign vestibular schwannoma resection, no neurofibromatosis, no prior radiosurgery

Intervention

Observation and reporting of sporadic sarcoma arising at prior schwannoma resection site