Unilateral Subtotal Adrenalectomy for Pheochromocytoma in Multiple Endocrine Neoplasia Type 2 Patients

In Brief Objective: To determine the best surgical strategy for pheochromocytoma in multiple endocrine neoplasia type 2 (MEN2) patients. Background: Pheochromocytomas occur in 50% to 60% of MEN2 patients, approximately half of them eventually develop bilateral disease. Unilateral subtotal adrenalectomy as primary surgery for pheochromocytoma in these patients may avoid or postpone the need for corticosteroid replacement therapy and the risk of Addisonian crisis, but is not yet widely accepted. Methods: We conducted a retrospective cohort study including 61 MEN2 patients with pheochromocytoma who were treated at the University Medical Center Utrecht between 1959 and 2010. Surgery was classified into 4 adrenalectomy groups: bilateral total, unilateral total, bilateral subtotal, and unilateral subtotal. Results: Primary surgery involved 22 bilateral total, 30 unilateral total, 2 bilateral subtotal, and 7 unilateral subtotal adrenalectomies. Twenty-one patients developed ipsilateral or contralateral recurrence after a median follow-up of 13.4 ± 10.8 years (range: 0.1–41.8). Unilateral total and unilateral subtotal adrenalectomy had similar rates of recurrence (P = 0.232) and survival time (5.5 versus 8.8 years; P = 0.170). Steroid replacement after bilateral total adrenalectomy led to complications in 8 patients. Reoperations for recurrence included unilateral total adrenalectomy in 12 patients, after which 10 needed steroid replacement (with complications in 3) and unilateral subtotal adrenalectomy in 5 patients, after which none needed replacement therapy. Ipsilateral recurrence after reoperation was similar between these groups. Conclusions: Unilateral subtotal adrenalectomy is a feasible surgical strategy for pheochromocytoma in MEN2 patients. It has comparable recurrence rates and eventually less complications of steroid replacement compared to unilateral total adrenalectomy. Pheochromocytomas occur in 50% to 60% of patients with multiple endocrine neoplasia type 2. Unilateral subtotal adrenalectomy as primary surgery for pheochromocytoma in multiple endocrine neoplasia type 2 patients avoids the need for corticosteroid replacement therapy and the risk of Addisonian crisis. According to our study, it is a feasible surgical strategy in terms of recurrence as well.