Phaeochromocytoma in Northern Ireland: A 21 year review

Objective: To report our experience of 41 patients with phaeochromocytoma. Design: Retrospective study. Setting: Teaching hospital, United Kingdom. Subjects: Forty-one patients who presented with phaeochromocytoma 1970-1991. Interventions: Removal of tumour (n = 38). Main outcome measures: Mortality, morbidity, and recurrence. Results: Thirty-four patients had sporadic tumours, five had the multiple endocrine neoplasia (MEN) type 2 syndrome, and two had non-MEN familial phaeochromocytoma. Thirty-six patients (88%) presented with symptoms of catecholamine excess, and 37 (90%) were hypertensive. The diagnosis was confirmed biochemically in 37. Tumours were located using computed tomography (n = 26), vascular studies (n = 11), and ultrasonography (n = 3). Thirty-eight patients had their tumours resected, of whom 10 (27%) developed complications. There were no postoperative deaths. Two patients were managed conservatively, and one died before diagnosis. Six patients developed recurrent tumours a mean of five years after the initial operation, and another patient had an inoperable tumour at initial diagnosis; four of these seven died from metastatic disease. Metaiodobenzylguanidine (MIBG) scans were positive in three of the patients who developed recurrences. Conclusion: Patients with phaeochromocytoma can now be operated on safely but prolonged follow-up is essential.