Interestingly, somatic mutations that result in a similar loss of function have been described in the human RIα locus (PRKAR1α) in patients with Carney complex, a familial multiple neoplasia syndrome characterized by cardiac and extracardiac myxomas, schwannomas, endocrine and gonadal tumors [48]–[50]. The gene discussed is PRKAR1A; the disease is schwannoma.