Death from phaeochromocytoma: lessons from a post-mortem survey

Despite advances in biochemical assessment and imaging, phaeochromocytoma remains a difficult diagnosis. Using the names of patients whose death certificate listed phaeochromocytoma as a cause of death, a retrospective survey of 62 deaths from phaeochromocytoma (48 benign, 14 malignant) was carried out. All deaths occurred between 1981 and 1989, so the pitfalls uncovered reflect recent practice. A substantial proportion presented with abdominal pain and vomiting, dyspnoea, left ventricular failure or hypotension rather than the classical symptoms. These presentations were more common in this autopsy series than in prospective series of consecutive patients. Diagnosis in the presence of classical symptoms was often delayed but, once it was made, elective excision was relatively safe. A personal or family history of symptoms suggesting inherited diseases associated with phaeochromocytoma was not always given due weight. Biochemical tests, particularly 24 hour urinary vanillyl mandelic acid, often gave contradictory results; the limits of their predictive power should be better appreciated. Anaesthesia and surgery in the presence of undiagnosed phaeochromocytoma was the cause of death in 16 of 62 cases. Recommendations to improve the accuracy of diagnosis are made.