A consecutive series of patients (68 females and 53 males) with pheochromocytoma (PC, n=110) or paraganglioma (PG, n=11) were treated at the Sahlgrenska University Hospital (1950-1997). During the observation period (15+/-6 years) 42 patients died versus 23.6 expected in the general population (P<0.001). There was no surgical mortality. Twenty patients died of cardiovascular disease, 11 of other tumors, and 7 of other diseases, but only 4 of PC/PG. The main causes of death in this regional series were cardiovascular diseases and tumor in a ratio of 1.3 versus 2.0 in the general Swedish population. Analysis of the mortality in all patients with clinically diagnosed PC (n=481, 259 women and 222 men) based on the National Cancer Registry (1957-1997) showed that the number of deaths in this cohort was 196 versus 153.4 expected (P<0.001). These patients had almost four times higher risk of dying of a tumor than did the general population (similar risk for females and males). There was no increased risk for cardiovascular death; in fact, the risk was lower than expected for men (22 vs. 38 expected). A second tumor diagnosed subsequent to PC occurred in 68 versus 31 expected. In men tumors of the liver and biliary tract and central nervous system and in women malignant melanoma and cervix carcinoma were most frequent. The results from the national series thus confirm an increased risk of a second tumor and increased tumor-related mortality in patients with PC.