Incidence and Clinical Presentation of Pheochromocytoma and Sympathetic Paraganglioma: A Population-based Study

Abstract Context Pheochromocytoma and sympathetic paraganglioma (PPGL) are rare catecholamine-secreting tumors but recent studies suggest increasing incidence. Traditionally, PPGL described to present with paroxysmal symptoms hypertension, existing data on clinical presentation of come from referral centers. Objective We aimed describe time trends in incidence a population-based study. Methods conducted nationwide retrospective cohort study previously validated 567 patients diagnosed Denmark 1977-2015. collected medical records geographic subcohort 192 patients. calculated age-standardized rates (SIRs) prevalence for the descriptive statistics subset data. Results SIRs increased 1.4 (95% CI 0.2-2.5) per million person-years 1977 6.6 4.4-8.7) 2015, corresponding 4.8-fold increase. The increase was mainly due incidentally found that were less than 4 cm older 50 years no or limited catecholamine excess. On December 31, 64.4 (CI 95% 57.7-71.2) inhabitants. Of data, 171 (89.1%) had unilateral pheochromocytoma, while (n = 13, 6.8%) multifocal 8, 4.2%) rare. Conclusion Incidence has 2015 “new” group presenting smaller few symptoms.