Phaeochromocytomas and Paragangliomas: A difference in disease behaviour and clinical outcomes. Issue 5 (4th September 2015)

• Record Type: Journal Article
• Authors: Ezzat Abdel‐Aziz, Tarek; Prete, Francesco; Conway, Gerard; Gaze, Mark; Bomanji, Jamshed; Bouloux, Pierre; Khoo, Bernard; Caplin, Martyn; Mushtaq, Imran; Smart, James; Kurzawinski, Tom R.
• Is Part Of: Journal of surgical oncology. Volume 112:Issue 5(2015:Oct. 01)
• Page Start: 486
• Page End: 491
• Publication Date: 2015-09-04
• Subjects: Cancer -- Surgery -- Periodicals; Neoplasms -- Periodicals; 616
• DOI: 10.1002/jso.24030
• Languages: English
• ISSNs: 0022-4790

Abstract

<abstract abstract-type="main" xml:lang="en"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="jso24030-sec-0001" sec-type="section"> <title>Background</title> <p>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.</p> </sec> <sec id="jso24030-sec-0002" sec-type="section"> <title>Methods</title> <p>A retrospective review was undertaken of all patients treated for these conditions at our centre between 1983 and 2012.</p> </sec> <sec id="jso24030-sec-0003" sec-type="section"> <title>Results</title> <p>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 (<italic>P</italic> < 0.001). More paragangliomas were malignant (14/23 vs 9/83, <italic>P</italic> < 0.0001), larger (9.17 ± 4.95 cm vs. 5.8 ± 3.44 cm, <italic>P</italic> = 0.001) and had a higher rate of conversion to open surgery (<italic>P</italic> = <0.01), more R2 resections, more postoperative complications and a longer hospital stay (<italic>P</italic> = 0.014). MIBG uptake in malignant paragangliomas was lower than in malignant phaeochromocytomas (36% vs. 100%, <italic>P</italic> = 0.002) and disease stabilisation was achieved in 29% and 86% of patients respectively. <sup>90</sup>Y‐DOTA‐octreotate had a 78% response rate in malignant paragangliomas.</p> </sec> <sec id="jso24030-sec-0004" sec-type="section"> <title>Conclusion</title> <p>The clinical differences between paragangliomas and phaeochromocytomas support the view that they should be considered as separate clinical entities. <italic>J. Surg. Oncol. 2015; 112:486–491</italic>. © 2015 Wiley Periodicals, Inc.</p> </sec> </abstract>