Clinical manifestations of pheochromocytomas and paragangliomas depending on the disease form and tumor characteristics: an analysis of a large patient cohort

Objective. To characterize the frequency of various symptoms of pheochromocytoma/paraganglioma (PPGL) considering the clinical form of the disease and main tumor characteristics. Dеsign and methods. The study included medical records of 254 patients examined and operated for PPGL with detailed information on disease symptoms, including data obtained through standardized questionnaires. Depending on the PPGL manifestations, all patients were divided into 4 groups according to the clinical manifestations. Results. In the cohort, the most common symptoms were hypertensive crises (55,1 %), a feeling of tachycardia (54,3 %), constant increase in blood pressure (40,6 %), headache (39,4 %), sweating (32,3 %), and general weakness (28,0 %). The following symptoms were more common (Fisher's exact test with the Friedman-Hamilton extension was used) in patients with paroxysmal and mixed forms of the disease in comparison with continuous and asymptomatic forms: sensation of tachycardia (64,1 % and 61,3 % versus 25,0 % and 9,4 % for the subgroups, respectively; p < 0,001), headache (51,3 % and 35,5 % versus 16,7 % and 15,6 %; p < 0,001), dizziness (17,1 % and 25,8 % versus 0,0 % and 6,3 %; p = 0,024), hand tremor (27,4 % and 15,1 % versus 0,0 % and 3,1 %; p = 0,002), fear and anxiety (23,9 % and 25,8 % versus 0,0 % and 6,3 %; p = 0,027), feeling of hot flashes / fever or chills (30,8 % and 20,4 % versus 0,0 % and 9,4 %; p = 0,010), sweating (38,5 % and 36,6 % versus 8,3 % and 6,3 %; p = 0,001). The patients with paroxysmal or mixed forms of the disease manifested with the highest number of complaints. The total number of complaints correlated with the maximum systolic and diastolic blood pressure (BP) values, but not with the usual BP values. The classic triad of PPGL symptoms (headache, sweating, tachycardia) was present in 11,8 % of patients. Conclusion. The article provides detailed data on the symptomatology of PPGL in general and for individual clinical forms of the disease. The obtained data indicate that the main driver of clinical symptoms in PPGL is the presence of paroxysms during the disease course. Comparing to previous years, PPGL manifestations became less pronounced over time, which is likely due to improvements in PPGL diagnosis and refined diagnostic algorithms for patients with adrenal incidentalomas.

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