January - March 2020, Volume 16, Issue 1

Cvasciuc IT, Gull S, Oprean R, Lim KH, Eatock F

Changing Pattern of Pheochromocytoma and Paraganglioma in a Stable UK Population

Acta Endo (Buc) 2020, 16 (1): 78-85
doi: 10.4183/aeb.2020.78

Context. Pheochromocytomas and paragangliomas (PCC/PGLs) are diagnosed variously with increasing incidence and changing clinical and pathology pattern. Objective. The aim was to further characterize PCC/PGLs in a stable population. Methods. A retrospective, single institution study analysed adrenalectomies for PCC/PGLs between January 2010 - January 2019. Demographics, symptoms, blood pressure, preoperative hormones, imaging, histology, hospital stay, complications and three subgroups [based on the modality of diagnosis - incidentaloma group (IG), genetic group (GG) and symptomatic group (SG)] were noted. Results. 86 patients included IG 51 (59.3%), GG 10 (11.62%) and SG 25 patients (29.06%). Incidence was 5.30 cases/1 million population. 33.34% of the IG had a delayed diagnosis with a mean interval of 22.95 months (4- 120 months). Females presented more often with paroxysmal symptoms (PS) (p=0.011). Patients with PS and classic symptoms were younger (p=0.0087, p=0.0004) and those with PS required more inotropes postoperatively (p=0.014). SG had higher preoperative hormone levels (p=0.0048), larger tumors (p=0.0169) and more likely females. GG are younger compared with those from the IG (p=0.0001) or SG (p= 0.178). Conclusion. Majority of patients had an incidental and delayed diagnosis. If symptomatic, patients are more likely to be young females with higher hormone levels and larger tumors.

Keywords: pheochromocytoma, paraganglioma, clinical pattern, incidence, PCC, PGL

Correspondence: Kah Heng Lim MD, Trauma and Orthopaedics, 59 Glendarragh, Belfast, BT4 2WB, United Kingdom of Great Britain and Northern Ireland, E-mail: kahheng.lim@rcsed.net