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Table 2 Incidence of MPNST in 12 tumour prone genetic conditions

From: Malignant peripheral nerve sheath tumours in inherited disease

Disease Cases at risk MPNST MPNST after radiation treatment Sex ratio M:F Median (range) age at diagnosis
NF1 1252 52 4 22:30 31 (13.3–77.2)
Schwannomatosis 181 3 0 2:1 26 (17–45)
NF2 920 2 2 1:1 25–34
BRCA1 2992* 0 0   
BRCA2 2735* 0 0   
TP53 221* 3 0 2:1 32 (1–33)
FAP 477 0 0   
MSH2 1054* 0 0   
MLH1 897* 0 0   
MSH6 178* 0 0   
vHL 87 0 0   
Gorlin syndrome 202 0 0   
  1. *Cases include first degree relatives of known mutation carriers.