PRKAR1A and neoplasm: This case affected a 14-year-old female with a history of the Carney complex in her mother.12 The Carney complex is characterized by germ line PRKAR1A mutations in the majority of patients.17 The model of fibrolamellar carcinogenesis as a tumor driven by activation of protein kinase A would be fulfilled in this patient’s case by loss of PRKAR1A function, which encodes a negative regulator of protein kinase A activity.