The role of adrenalectomy in the management of pheochromocytoma: the experience of a Portuguese referral center

Abstract

Abstract Purpose: Pheochromocytoma is a rare neuroendocrine tumor. Despite the low incidence, these tumors are of indisputable importance. This study aimed to analyze the management of pheochromocytoma in a referral center, with an emphasis on the minimally invasive adrenalectomy, which is the preferred therapeutic approach. Methods: A retrospective analysis was performed on a cohort of patients diagnosed with pheochromocytoma who underwent adrenalectomy between January 2013 and December 2022. Clinical data including demographics, timelines, symptomatology, comorbidities, biochemical markers, genetic testing, surgical details, and follow-up outcomes, were collected and analyzed. Results: The cohort included 44 patients, predominantly women (52,3%), with a median age of 53,4 years (range 13-83). Most patients exhibited paroxysmal symptoms suggesting catecholamine excess. Documented hypertension was the most frequent (86,4%), along with glucose anomalies (40,9%) and anxiety disorder (31,8%). Genetic testing was performed in 36 (81,8%) patients and 14 (38,9%) revealed a positive result, predominantly RET pathogenic variant. Laparoscopic surgery was performed in 34 (77,3%) patients, showing significantly shorter operative time (2,5 hours vs. 4,3 hours, p<0,001) and fewer complications (23,5% vs 77,8%, p=0,008). Postoperative complications occurred in 36,4% of the patients, mostly mild (grade I, 56,3%), with no mortality. SDHB pathogenic variant associated with recurrent disease (p=0.006). One-year follow-up reported recurrence in 9.1% and metastasis in 2,3%. Conclusions: Adrenalectomy demonstrated high safety and effectiveness. This study exhibited a higher rate of genetic testing referral than other studies. Despite past advances, there is still a need for further studies to establish protocols and evaluate new techniques.