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.


CONCLUSIONS
The clinical differences between paragangliomas and phaeochromocytomas support the view that they should be considered as separate clinical entities.
Centre for Endocrine Surgery, University College London Hospital and Great Ormond Street Hospital, London, UK.|Department of General Surgery, University of Alexandria, Egypt.
Tarek
T
Ezzat Abdel-Aziz
Centre for Endocrine Surgery, University College London Hospital and Great Ormond Street Hospital, London, UK.
Francesco
F
Prete
Departments of Oncology, Endocrinology and Nuclear Medicine, University College London Hospital, London, UK.
Gerard
G
Conway
Departments of Oncology, Endocrinology and Nuclear Medicine, University College London Hospital, London, UK.
Mark
M
Gaze
Departments of Oncology, Endocrinology and Nuclear Medicine, University College London Hospital, London, UK.
Jamshed
J
Bomanji
Department of Endocrinology, Oncology and Neuroendocrine Unit, Royal Free Hospital, London, UK.
Pierre
P
Bouloux
Department of Endocrinology, Oncology and Neuroendocrine Unit, Royal Free Hospital, London, UK.
Bernard
B
Khoo
Department of Endocrinology, Oncology and Neuroendocrine Unit, Royal Free Hospital, London, UK.
Martyn
M
Caplin
Centre for Endocrine Surgery, University College London Hospital and Great Ormond Street Hospital, London, UK.
Imran
I
Mushtaq
Centre for Endocrine Surgery, University College London Hospital and Great Ormond Street Hospital, London, UK.
James
J
Smart
Centre for Endocrine Surgery, University College London Hospital and Great Ormond Street Hospital, London, UK.
Tom R
TR
Kurzawinski
D019797:3-Iodobenzylguanidine / Q000493:pharmacokinetics; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000000981:diagnostic imaging / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D000328:Adult; D000368:Aged; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006571:Heterocyclic Compounds / Q000493:pharmacokinetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009942:Organometallic Compounds / Q000493:pharmacokinetics; D010235:Paraganglioma / Q000000981:diagnostic imaging / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D010673:Pheochromocytoma / Q000000981:diagnostic imaging / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D011379:Prognosis; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals / Q000493:pharmacokinetics; D012189:Retrospective Studies; D015996:Survival Rate; D014018:Tissue Distribution; D014057:Tomography, X-Ray Computed; D055815:Young Adult
D006571:Heterocyclic Compounds; D009942:Organometallic Compounds; D019275:Radiopharmaceuticals; C113625:yttrium(III)-1,4,7,10-tetraazacyclotetradecane-N,N',N'',N'''-tetraacetic acid; D019797:3-Iodobenzylguanidine
adrenalectomy; laparoscopy; malignancy; paraganglioma; phaeochromocytoma; radio-targeted therapy