Outcome of Partial Adrenalectomy in MEN2 Syndrome: Personal Experience and Systematic Review of Literature

Abstract

Background: Partial adrenalectomy (PA) is an alternative option to total adrenalectomy for the treatment of hereditary pheochromocytoma (PHEO) to preserve cortical function and avoid life-long steroid replacement. The aim of this review is to summarize current evidence in terms of clinical outcome, recurrence, and corticosteroid therapy implementation after PA for MEN2-PHEOs. Material and Methods: From a total of 931 adrenalectomies (1997–2022), 16 of the 194 patients who underwent surgical treatment of PHEO had MEN2 syndrome. There were six patients scheduled for PA. MEDLINE®, EMBASE®, Web of Science, and Cochrane Library were searched for English studies from 1981 to 2022. Results: Among six patients who underwent PA for MEN2-related PHEO in our center, we reported two with bilateral synchronous disease and three with metachronous PHEOs. One recurrence was registered. Less than 20 mg/day Hydrocortison therapy was necessary in 50% of patients after bilateral procedures. Systematic review identified 83 PA for MEN2-PHEO. Bilateral synchronous PHEO, metachronous PHEO and disease recurrence were reported in 42%, 26%, and 4% of patients, respectively. Postoperative steroid implementation was necessary in 65% of patients who underwent bilateral procedures. Conclusions: PA seems to be a safe and valuable option for the treatment of MEN2-related PHEOs, balancing the risk of disease recurrence with the need for corticosteroid therapy.