GénOncoPGxBio
Back
Autosomal dominantPubMedPenetrance update

Survival and prognosis of neurofibromatosis type 1-associated malignant peripheral nerve sheath tumours: a systematic review and meta-analysis

Handrup MM, Aggerholm-Pedersen N, Thomasen SB, et al. — Orphanet J Rare Dis 2026 · June 2026
Relevance score
6/10
Disease / domain
Neurofibromatosis type 1 — malignant peripheral nerve sheath tumours (MPNST)
Source
PubMed
PMID 42310813
Share on LinkedIn

Gene / mechanism

Survival and prognosis of NF1-associated MPNST — meta-analysis

Summary

A systematic review and meta-analysis synthesizes survival and prognostic data for malignant peripheral nerve sheath tumours (MPNST) associated with neurofibromatosis type 1 (NF1). MPNST represent the most serious malignant complication of NF1 with a severe prognosis. This meta-analysis provides updated survival estimates and identifies prognostic factors, useful for genetic counseling and follow-up of NF1 carriers.

Synthesis written by Geno'X. For the full original abstract, please refer to the source publication.

Analysis

This meta-analysis fills a gap in quantifying MPNST risk for NF1 carriers, essential information during genetic counseling. The rarity of these tumors makes such a synthesis all the more valuable. Updated survival data allow more precise information to families about the prognosis of this feared complication.

Why this score?

Impact 1/3Evidence 3/3Novelty 1/2Sample 1/1Publication 0/1

Clinical impact: 1/3 · Evidence strength: 3/3 · Novelty: 1/2 · Sample size: 1/1 · Publication status: 0/1 → Total: 6/10

Keywords

neurofibromatosis type 1NF1MPNSTprognosissurveillance
Weekly report in your inbox

Every Wednesday · Annotated selection · Free · Unsubscribe anytime