Phaeochromocytomas and paragangliomas: A difference in disease behaviour and clinical outcomes.

BACKGROUND: Phaeochromocytomas and paragangliomas arise from the same chromaffin cell, but evidence suggests they do not represent a single clinical entity. The aim of this study was to compare clinical presentations, outcomes of surgical and oncological treatments and survival in patients with phaeochromocytomas and paragangliomas.

METHODS: A retrospective review was undertaken of all patients treated for these conditions at our centre between 1983 and 2012.

RESULTS: One hundred and six patients (88 adults, 18 children) with phaeochromocytoma (n = 83) or paraganglioma (n = 23) were studied. Catecholamine symptoms and incidentalomas were the main presentations in phaeochromocytoma patients (67% and 17%) respectively, but in those with paragangliomas pain (39%) was more common (P < 0.001). More paragangliomas were malignant (14/23 vs 9/83, P < 0.0001), larger (9.17 ± 4.95 cm vs. 5.8 ± 3.44 cm, P = 0.001) and had a higher rate of conversion to open surgery (P = <0.01), more R2 resections, more postoperative complications and a longer hospital stay (P = 0.014). MIBG uptake in malignant paragangliomas was lower than in malignant phaeochromocytomas (36% vs. 100%, P = 0.002) and disease stabilisation was achieved in 29% and 86% of patients respectively. (90) Y-DOTA-octreotate had a 78% response rate in malignant paragangliomas.

CONCLUSION: The clinical differences between paragangliomas and phaeochromocytomas support the view that they should be considered as separate clinical entities.