Portrait of a Series of Patients with Pheochromocytoma/Paraganglioma from a Reference Center in Brazil: Relevance of Prior Background Features

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PURPOSE: Pheochromocytomas (Pheo) and paragangliomas (PGL) are catecholamine-secreting tumours, whose functionality is confirmed by elevated plasma (Pl) and/or 24-h urinary (Ur) metanephrines (MN). Relevance of prior background features were reviewed in a large cohort of patients with Pheo and PGL. MATERIAL AND METHODS: We reviewed clinical, hormonal, and imaging aspects of 116 patients studied prospectively: 93 Pheo; 22 PGL; one Pheo plus PGL. RESULTS: Twenty-five % PPGL were discovered incidentally. Systemic arterial hypertension (SAH) was present in 81% (43% on stage 3), whereas 9.5% were prehypertensive and 9.5%, normotensive. SAH plus paroxysms occurred in 31 (32.9%) patients, being exclusively sustained in the remaining; 26 (28%) had resistant SAH. Orthostatic hypotension was seen in 65% of patients. Pl/Ur MN and normetanephrine (NMN) were compared to those of a positive (56 functioning PPGL) and a negative control group (654 subjects with normal MN/NMN). Total and fractionated Ur MN were elevated in 94% PPGL patients. Cut-off values of 885 mcg/24-h for Ur MN, and of 1.5 nmol/L for Pl MN identified functioning lesions with 100%/100% sensitivity and 93%/97% specificity, respectively. MRI detected 56% right-side Pheo, 25% on the left, and 19% bilateral; PGL were 56.5% (13/23) retroperitoneal and 43.5%, cervical (10/23). Right-side Pheo were larger (5.8 cm) than left-side ones (3.7 cm), but retroperitoneal (6.5 cm) and neck PGL (6.9 cm) were similar. Tumour size positively correlated with total Ur MN. CONCLUSIONS: in this large cohort of PPGL patients we highlighted relevant aspects of SAH, the frequently overlooked manifestation of orthostatic hypotension, common incidental presentation, significant tumour size/hormonal production.

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