Transformation of vestibular schwannomas into malignant peripheral sheath tumors: a systematic review and pooled analysis of patient characteristics and survival outcomes

Transformation of vestibular schwannoma (VS) into malignant peripheral nerve sheath tumor (MPNST) is rare and poorly characterized. We have synthesized available evidence from the literature to define clinical features, transformation patterns, management strategies, and survival outcomes in patients with MPNST after histologically confirmed transformation from VS.

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No immediate practice change is warranted; this is a rare, poorly understood complication, and the review is primarily informative for neurotology specialists managing vestibular schwannoma patients long-term.

Understanding the rare but life-threatening malignant transformation of vestibular schwannomas is critical for long-term surveillance protocols and counseling patients about worst-case disease progression.

1. 01Malignant transformation of vestibular schwannomas into MPNSTs is extremely rare but carries very poor survival outcomes.
2. 02Systematic review and pooled analysis design provides the highest-level summary of existing case data.
3. 03Patient characteristics and survival data are pooled to identify shared risk factors or clinical patterns.
4. 04Findings are relevant primarily to neurotologists and skull-base surgeons managing complex schwannoma cases.
5. 05Prior radiation therapy may be a risk factor for malignant transformation, consistent with known MPNST biology.

PMID

42599576

DOI

10.1007/s11060-026-05722-0.

Journal

Journal of Neuro-Oncology

Publication type

systematic_review

Evidence level

1a

Population

Patients with documented malignant transformation of vestibular schwannoma into MPNST (case-level pooled data from published literature)

Intervention

Systematic review of malignant transformation of vestibular schwannomas