Clinical Presentation and Perioperative Management of Pheochromocytomas and Paragangliomas: A 4-Decade Experience

Abstract

Purpose Latin American reports on pheochromocytomas and paragangliomas (PPGL) are scarce. Recent studies demonstrate changes in clinical presentation and management of these patients. Herein, we assessed the main characteristics of PPGL patients in our academic center over the last four decades. Methods Demographic, clinical, biochemical and perioperative data from 105 PPGL patients were retrospectively and prospectively collected over the 1980-2019 period. Data were organized into four time periods by decades. Results Age at diagnosis, gender, tumor size and percentage of bilaterality, percentage of paragangliomas and metastases remained stable across the four decades. The proportion of genetic testing and incidentalomas increased in recent decades (all p<0.001). Therefore, we compared PPGLs diagnosed as incidentalomas (36%) to those clinically suspected (64%). Incidentalomas had fewer adrenergic symptoms (38 vs. 62%; p<0.001), lower rates of hypertension (64 vs. 80%; p=0.01) and hypertensive crisis (28 vs. 44%; p=0.02); also they had lower functionality (79 vs. 100%; p=0.01) and lower catecholamines levels (8.4 vs. 12.5-fold above upper cut-offs; p=0.04). Regarding management of all PPGLs over the decades, we observed significant increases in both perioperative doxazosin dose (p=0.003) and laparoscopic approach rates (p<0.001), along with a decrease in the length of hospital stays (p=0.007). Conclusions We observed a change in the clinical presentation of PPGL in recent decades, with a marked increase in incidental cases and milder symptoms. The implementation of a multidisciplinary program for adrenal disorders in our institution has translated into more timely diagnoses, more genetic testing, and improvements in perioperative management.

Advances in the Diagnosis and Treatment of Pheochromocytomas and Paragangliomas in the Era of Personalized Genetic Diagnostic

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