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 |