title: Long-term outcomes of surgical treatment for hereditary pheochromocytoma.
abstract: BACKGROUND
  The ideal surgical management of hereditary pheochromocytomas includes planning for a potential metachronous bilateral presentation and the possibility of lifelong steroid dependence if bilateral adrenalectomy is needed. An intact and viable cortical remnant after bilateral pheochromocytoma resection can eliminate the necessity for steroid dependency, but can increase the risk of pheochromocytoma recurrence.
  
  
  METHODS
  We retrospectively reviewed outcomes of all patients with a diagnosis of hereditary pheochromocytomas treated at our tertiary cancer institution from 1962-2011, with subset analysis of patients undergoing a cortical-sparing procedure in the setting of bilateral adrenalectomy.
  
  
  RESULTS
  Of the ninety-six patients who underwent adrenalectomy for hereditary pheochromocytomas, 47 presented with bilateral disease. In 15 of the 49 patients (30%) who originally underwent unilateral adrenalectomy, pheochromocytoma developed in the contralateral gland at a median of 8.2 years (range 1 to 20 years) after the initial diagnosis. There were 4 recurrences in 55 cortical-sparing remnants (7%) and 3 recurrences in the adrenal bed after 101 intended total adrenal resections (3%) (p = 0.24). Total bilateral adrenalectomy was performed in 25 patients and acute adrenal insufficiency developed in 5 (20%) of those patients. An intended cortical-sparing adrenalectomy was performed in 39 patients and acute adrenal insufficiency developed in 1 (3%). Of these patients with adequate follow-up, 21 of 27 (78%) were steroid independent at 3-year follow-up. Sex, median age, adrenal vein preservation, metachronous adrenal resection, and bilateral cortical-sparing procedures did not predict steroid independence at 3 years.
  
  
  CONCLUSIONS
  Cortical-sparing adrenalectomy avoids long-term corticosteroid dependence in the majority of patients with hereditary pheochromocytoma with minimal risk of acute adrenal insufficiency. Recurrence occurs in approximately 7% of adrenal remnants.
authors: Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX 77030-4008, USA. eggrubbs@mdanderson.org
authors: Elizabeth G
authors: EG
authors: Grubbs
authors: Thereasa A
authors: TA
authors: Rich
authors: Chaan
authors: C
authors: Ng
authors: Priya R
authors: PR
authors: Bhosale
authors: Camilo
authors: C
authors: Jimenez
authors: Douglas B
authors: DB
authors: Evans
authors: Jeffrey E
authors: JE
authors: Lee
authors: Nancy D
authors: ND
authors: Perrier
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000235:genetics / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D005820:Genetic Testing; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D010673:Pheochromocytoma / Q000235:genetics / Q000601:surgery*; D011183:Postoperative Complications; D012189:Retrospective Studies; D016896:Treatment Outcome