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Phaeochromocytomas and Paragangliomas: A difference in disease behaviour and clinical outcomes
Author(s) -
Ezzat AbdelAziz Tarek,
Prete Francesco,
Conway Gerard,
Gaze Mark,
Bomanji Jamshed,
Bouloux Pierre,
Khoo Bernard,
Caplin Martyn,
Mushtaq Imran,
Smart James,
Kurzawinski Tom R.
Publication year - 2015
Publication title -
journal of surgical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.201
H-Index - 111
eISSN - 1096-9098
pISSN - 0022-4790
DOI - 10.1002/jso.24030
Subject(s) - medicine , paraganglioma , pheochromocytoma , disease , retrospective cohort study , overall survival , surgery
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. J. Surg. Oncol. 2015; 112:486–491 . © 2015 Wiley Periodicals, Inc.