Table. Median Age of Onset and Prevalence of Characteristic Tumors in 215 Patients With BAP1 Syndrome.
| Tumor | Cases, No. | Estimated Penetrance, %a | Median Age of Diagnosis in the Literature, y | Median Age of Diagnosis in Our Series, y | Median Age of Diagnosis in General Population, y |
|---|---|---|---|---|---|
| Uveal melanoma | 60 | 28.0 | 53 | 59 | 61 |
| Mesothelioma | 48 | 22.0 | 56 | 46 | 74 |
| Cutaneous melanoma | 38 | 18.0 | 41 | 43 | 61 |
| MBAITs | 36 | 17.0 | 32 | 31 | 24 |
| Renal cell carcinoma | 20 | 9.0 | 47 | 51 | 64 |
| Basal cell carcinoma | 14 | 6.5 | 52 | 41 | 75 |
Abbreviation: MBAITs, melanocytic BAP1–associated intradermal tumors.
a
The estimated penetrance is based on the prevalence of each tumor reported in BAP1 patients identified in the literature and in our series.