Background: Adrenal pheochromocytoma is potentially lethal if undetected and is associated with long-term morbidity.

Methods: Records of patients (11 men, 18 women) with confirmed pheochromocytoma were studied with respect to clinicopathological features and outcome.

Results: Pheochromocytoma was diagnosed in 4 of 8,486 (0.05%) autopsies and accounted for 3 of the 4 immediate causes of death. Operative mortality occurred in 1 patient with undiagnosed tumor. Hypertensive-related complications occurred in 6 patients before diagnosis. Malignancy was documented in 7 patients with distant metastases (n = 4) or locally invasive tumors (n = 3). During a median follow-up of 4 years, 23 patients are alive and free of disease (79%). Four of the 6 patients (67%) who died had distant metastases compared with 2 of 23 patients (9%) without distant metastases (P = 0.04). Persistent hypertension and diabetes requiring treatment were present in 8 (35%) and 4 (17%) of 23 patients respectively.

Conclusions: Fatal complications preceded diagnosis in a significant proportion of patients with pheochromocytoma. The presence of distant metastases was associated with poor survival.