The role of the 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 (52,27%), with a median age of 53,39 years (range 13–83). Most of patients exhibited paroxysmal symptoms suggesting catecholamine excess. Documented hypertension was the most frequent (86,36%), along with glucose anomalies (40,01%) and anxiety disorder (31,82%). Genetic testing was performed in 36 (81,81%) patients and 14 (38,88%) revealed a positive result, predominantly RET pathogenic variant. Laparoscopic surgery was performed in 34 (79,07%) patients, showing significantly shorter operative time (2,5 hours vs. 4,25 hours, t-test p < 0,001) and fewer complications (23,53% vs 77,78%, p = 0,008). Postoperative complications occurred in 36,36% of the patients, mostly mild (grade I, 56,25%), with no mortality. SDHB pathogenic variant correlated with both recurrent and metastatic disease (p = 0.006). One-year follow-up reported 9.09% recurrence and 6.82% metastasis. Conclusions Adrenalectomy demonstrated a 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.