LFS is closely associated with mutations in the TP53 gene, which plays a central role in controlling cell division and cell death. When TP53 is mutated, the body’s ability to repair cell damage is significantly impaired, leading to a greatly increased susceptibility to many types of cancer.
In LFS, ACC tends to occur during childhood. About 6–13% of children with LFS develop ACC, often between the ages of 1 and 4.