Multicenter Study on Cardiovascular and Metabolic Complications in Patients With Biochemically Silent Pheochromocytomas and Paragangliomas

Multicenter Study on Cardiovascular and Metabolic Complications in Patients With Biochemically Silent Pheochromocytomas and Paragangliomas

The aim of the study is to characterise the cardiovascular and metabolic complications pre- and post-surgery of patients with biochemically negative PPGL and to compare them with normal individuals and patients with secreting PPGLs age and sex matched.

Pheochromocytomas (PHEO) and Paragangliomas (PGL) represent a group of tumors associated with increased cardiometabolic morbidities and mortality. The secretory components of PHEOs/PGLs are well-established risk factors for cardiovascular events and the Endocrine Society Clinical Practice Guidelines have implemented specific instructions for their management and their follow-up. However, data regarding the cardiovascular risk in PHEOs and PGLs asymptomatic patients with the normal biochemical profile are sparse and debating. The incidence of cardiovascular complications and the long-term morbidity in this group of patients is not well-characterized complicating the therapeutic approach or the necessity of pre-operative a-blockade as well as the optimal follow-up, regarding the duration and the need for cardiovascular examination.

Aim Primary outcome v To characterize the cardiovascular and metabolic complications of patients with biochemically negative PPGLs and to compare them with individuals age and sex-matched with normal adrenals (controls) as well as with patients with secreting PHEOs/PGLs.

Secondary outcome v To study also the sub-mentioned parameters post-treatment

Inclusion Criteria:

• All PHEOs/PGLs patients should be asymptomatic, with confirmed histopathological analysis for those who had surgery or with positive specific imaging functional studies (MIBG, 68Ga DOTATATE, Octreoscan, Tektrotyde)
• Data about the plasma or urine levels of catecholamines, normetanephrines, metanephrines and methoxytyramine (for PGL) levels should be available for every patient and should be lower than the upper normal values measured with LC-MS (supplementary data about CgA, NSE levels can be also included)
• Available data for the "required" cardiovascular paraclinical or metabolic parameters should be available pre-surgery or at the time of diagnosis or at follow-up for the "not operable" tumors as well as post any treatment

Exclusion Criteria:

• Patients with ambiguous diagnosis of PHEOs/PGLs (absence of histological report and non-specific imaging findings)
• Catecholamines, normetanephrines and metanephrines (and methoxytyramine levels for PGL) levels higher than the upper limit range
• No available data of the required parameters