Table 5. Standardized incidence ratios (SIRs) for malignancies in Japan-resident Werner syndrome patients.*** .
tumor | observed**** | expected**** | SIR | 95% CI |
melanoma of skin | 9 | 0.17 | 53.5* | (24.5, 101.6) |
meningioma | 10 | 0.28 | 36.2* | (17.3, 66.5) |
soft tissue | 11 | 0.35 | 31.1* | (15.5, 55.7) |
bone | 9 | 0.33 | 27.1* | (12.4, 51.4) |
thyroid | 14 | 1.57 | 8.9* | (4.9, 15.0) |
leukemia | 5 | 2.47 | 2.0 | (0.66, 4.7) |
all sites** | 90 | 103.81 | 0.87 | (0.70, 1.07) |
statistically significant result (p<0.05).
includes benign meningiomas diagnosed prior to 1988, but excludes non-melanoma skin neoplasms.
for patients with high WS diagnostic confidence (1965–2009).
relative to Osaka, Japan population, 1965–2009.
note: analysis conditioned on a WRN pathogenic allele frequency of q = 0.0037.