title: Unilateral subtotal adrenalectomy for pheochromocytoma in multiple endocrine neoplasia type 2 patients: a feasible surgical strategy.
abstract: 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.
authors: Department of Surgical Oncology and Endocrine Surgery, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: Anouk
authors: A
authors: Scholten
authors: Gerlof D
authors: GD
authors: Valk
authors: Dionne
authors: D
authors: Ulfman
authors: Inne H M
authors: IH
authors: Borel Rinkes
authors: Menno R
authors: MR
authors: Vriens
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000401:mortality / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D000328:Adult; D015331:Cohort Studies; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D008297:Male; D008875:Middle Aged; D018813:Multiple Endocrine Neoplasia Type 2a / Q000601:surgery*; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000601:surgery; D010673:Pheochromocytoma / Q000401:mortality / Q000601:surgery*; D011183:Postoperative Complications / Q000097:blood; D012086:Reoperation; D012189:Retrospective Studies; D016019:Survival Analysis; D055815:Young Adult
chemical_list: D006854:Hydrocortisone