Among 6 studies of pediatric PPGL patients with MEN-2 due to a RET mutation, ten patients were identified (representing 0.6-3.3% of each cohort except for 13.0% in one study), all with PCC Age at PCC diagnosis ranged from 14-20 years; eight patients had bilateral or recurrent disease, and no patients were reported to have metastatic disease (2, 4, 5, 7, 8, 29). The gene discussed is RET; the disease is multiple endocrine neoplasia type 2.