This condition is characterized by autosomal dominant inheritance, early onset and a diverse array of cancers, including sarcomas, brain tumors, leukemias, adrenocortical tumors and breast cancer.40 Similar to inherited RB, the mutation in Li-Fraumeni syndrome results in the inactivation of a tumor suppressor gene (p53). The gene discussed is TP53; the disease is Li-Fraumeni syndrome.