Definition

A rare inherited disorder that is caused by mutations (changes) in the BAP1 gene. Having this mutation increases the risk of developing certain types of cancerous tumors of the skin, eye, kidney, and mesothelium (the tissue that lines the chest and abdomen). The cancers tend to develop at an early age, are often fast-growing, and are more likely to spread to other parts of the body. Benign (not cancer) tumors of the skin may also occur. Not all people who have a mutation in the BAP1 gene will develop tumors.