Endocrine Abstracts

Context: Pheochromocytomas and paragangliomas (PPGLs) are rare tumours with potentially harmful cardiovascular complications. Traditionally, they were detected in patients with symptoms and signs of catecholamine excess or during genetic surveillance. However, according to recent reports, PPGLs are increasingly being diagnosed in the setting of incidentally discovered adrenal masses.

Aim: To assess the initial clinical presentation of our PPGL patients with emphasis on the characteristics of the incidental subgroup.

Methods: We retrospectively reviewed our cohort of patients diagnosed with and operated on PPGLs between January 2005 and December 2020 at the national tertiary care centre. The diagnosis was pathohistologically verified.

Results: We included 126 patients (68 women) with PPGL, mean age 55 years (range 16-80), mean BMI 26.1 (range 17.3-45.5). Most tumours were located in the adrenal (92%; right 52%, bilateral 2%), the remaining minority being paragangliomas (8%). Tumour size ranged from 10 to 121 mm (mean 47 mm). Overall, 80 PPGLs (63%) presented incidentally, 39 (31%) with adrenergic symptoms and signs, and 7 (6%) were diagnosed via genetic screening. Incidentally detected patients were older (mean age 58 years) on average than symptomatic individuals (52 years) and patients with hereditary syndromes (35 years; P<0.001). Annual number of PPGLs has been increasing over the studied period from about five to about 10. The presenting age has significantly increased since 2010 (mean 57 vs 48 years; P=0.01) in parallel with the proportion of patients with incidentalomas (69% vs 47%; P=0.03). However, targeted clinical assessment revealed that 48% of the incidentaloma group had at least one of the typical clinical manifestations (e.g. headache, palpitations, perspiration, pallor), and 37% had stage 2 or 3 hypertension. Genetically detected patients exhibited statistically significantly lower levels of urinary and plasma metanephrines, while values in symptomatic and incidentaloma patients were similar and varied widely. Five tumours appeared nonfunctional (2 hereditary and 3 incidental PPGLs). Imaging was mostly done by CT (120 patients or 94%). The density of the adrenal lesions ranged from 11 to 50 Hounsfield units. Nuclear medicine procedures were performed in 113 patients (89%). MIBG scintigraphy was used in 93% of these cases, the others had PET with positive findings in 91% overall.

Conclusion: Our observations are in line with recent reports that the majority of PPGLs are diagnosed incidentally. As a significant proportion of these patients were not truly asymptomatic, awareness of the clinical presentation of PPGLs still seems lifesaving.