BAP1-TPDS is inherited in an autosomal dominant manner, which means that each child of an affected person has a 50% risk to inherit the condition. However, it appears that not everyone with a mutation will develop a tumor, and the types of BAP1-related tumors can vary among different members of the same family. To date, most individuals diagnosed with the BAP1-TPDS inherited it from a parent, which means that the person’s siblings and other relatives are also at risk to have the mutation. The proportion of BAP1-TPDS caused by a new mutation (i.e. not present in either parent) is unknown. Once a BAP1 mutation has been identified, other family members can be tested and prenatal testing for a pregnancy at risk for BAP1-TPDS is also possible. Genetic counseling is strongly recommended for all individuals and families found to have a BAP1 mutation.
