Pheochromocytomas and paragangliomas

Purpose of review Great progress has been made in understanding the genetic and molecular basis of pheochromocytoma and paragangliomas (PPGLs). This review highlights the new standards in the diagnosis and management of pediatric PPGLs. Recent findings The vast majority of pediatric PPGLs have an associated germline mutation, making genetic studies imperative in the work up of these tumors. Somatostatin receptor-based imaging modalities such as Ga-68-DOTATATE and Cu-64-DOTATATE are shown to have the greatest sensitivity in pediatric PPGLs. Peptide receptor radionuclide therapies (PRRTs) such as Lu-177-DOTATATE are shown to have efficacy for treating PPGLs. Summary Genetics play an important role in pediatric PPGLs. Advances in somatostatin receptor-based technology have led to use of Ga-68-DOTATATE and Cu-64-DOTATATE as preferred imaging modalities. While surgery remains the mainstay for management of PPGLs, PRRT is emerging as a treatment option for PPGLs.