Walpole S, et al. - Researchers attempted to better characterize the genotypes and phenotypes associated with the BRCA1-associated protein-1 (BAP1) tumor predisposition syndrome (BAP1-TPDS), which is a hereditary tumor syndrome, given that it is important to acquire improved understanding of the BAP1-TPDS to ensure appropriate clinical management of BAP1 germline variant carriers and their families. They analyzed data for 106 published and 75 unpublished BAP1 germline variant-positive families worldwide. The core tumor spectrum associated with the BAP1-TPDS was corroborated by the collated data. In addition, data revealed that this phenotype could be seen in some families carrying missense variants. Families of variant carriers had a variety of noncore BAP1-TPDS -associated tumors. For all tumors combined, mesothelioma, cutaneous melanoma, and nonmelanoma skin cancer, lower median ages of onset of core tumor types were observed in null vs missense variant carriers.