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

Authors

Othman Bin-Alamer, Muhammad Ammar Haider, Kishore Balasubramanian, Adi Mittal, Sarath Pathuri, David Botros, Zachary C Gersey, Hussam Abou-Al-Shaar, Robert C Rennert, Sarah T Menacho, William T Couldwell

Published in

Journal of neuro-oncology. Volume 179. Issue 1. Aug 14, 2026. Epub Aug 14, 2026.

Abstract

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.
A PRISMA-guided systematic review of PubMed, Scopus, and Google Scholar databases identified case reports and case series reporting histologically confirmed VSs transforming to MPNSTs with clinical and outcome data. Demographic, clinical, treatment, and survival variables were extracted and analyzed.
Twenty-one studies comprised 24 patients (7 male) with MPNSTs (median age 52 years; 8% with neurofibromatosis 2-related schwannomatosis). The median interval from VS to MPNST diagnosis was 54.0 months; 75% of patients had prior radiation-based therapy for VS. The transformation interval was significantly shorter in patients with spontaneous transformation compared with those whose transformation occurred after radiation (7.2 vs. 84.0 months; p = 0.001). All patients underwent resection after their tumors transformed into MPNST; 42% received adjuvant radiation-based therapy. Median overall survival was 11.0 months, and adjuvant radiation-based therapy was associated with longer overall survival (15.6 vs. 4.0 months; p < 0.001) in all patients with MPNST. Age at diagnosis, sex, tumor size, tumor laterality, p53 status, and hydrocephalus occurrence were not associated with a statistically significant difference in overall survival. Recurrence or progression occurred in 60% of evaluable patients.
Once transformation of VS into MPNST occurs, survival prognosis is bleak; however, these results indicate that early surgical intervention and adjunctive radiation-based therapy for MPNST may offer survival benefits despite high recurrence risk.

PMID:
42599576
Bibliographic data and abstract were imported from PubMed on 15 Aug 2026.

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