Partial adrenalectomy for pheochromocytoma in the multiple endocrine neoplasia type 2 population: Time to reconsider?

Abstract

Background: Adrenalectomy is considered the standard of care for multiple endocrine neoplasia type 2–related pheochromocytomas. Recently, partial adrenalectomy has been suggested as an alternative to prevent adrenal insufficiency in hereditary pheochromocytoma. Nevertheless, this comes with the risk of ipsilateral recurrence. Therefore, we aimed to determine the incidence of ipsilateral recurrence after partial adrenalectomy and assess the proportion of prevented adrenal insufficiency due to partial adrenalectomy in cases of bilateral disease. Methods: A retrospective, multicenter cohort study was conducted between 1977 and 2022 via a standardized medical record review. The primary outcome was the incidence of ipsilateral recurrence after partial adrenalectomy compared with total adrenalectomy. The secondary outcomes included disease-free survival and incidence of adrenal insufficiency and crisis after bilateral surgery. Results: In 50 patients with multiple endocrine neoplasia type 2, a total of 88 adrenalectomies were performed, including 23 partial adrenalectomies and 65 total adrenalectomies. After a median follow-up after last surgery of 8.3 years, 7 (30.4%) of the partial adrenalectomies and 2 (3.1%) of total adrenalectomies had recurrent disease (P < .001, χ2 test). The median time to recurrence was 4 and 21 years after partial adrenalectomy and total adrenalectomy, respectively. After bilateral surgery with at least partial adrenalectomy on one side, 50.0% of patients did not require corticosteroids. Conclusion: These data show that there is a considerable risk of ipsilateral recurrent disease in the short term after partial adrenalectomy for multiple endocrine neoplasia type 2–related pheochromocytoma with a 50% chance of adrenal insufficiency in cases of bilateral surgery. Therefore, we doubt whether partial adrenalectomy should be preferred for multiple endocrine neoplasia type 2–associated pheochromocytoma. We propose total adrenalectomy as the standard technique and partial adrenalectomy only for selected cases.