title: Surgical management of hereditary pheochromocytoma.
abstract: BACKGROUND
  Surgical treatment of hereditary pheochromocytoma remains controversial because of the need for lifelong corticosteroid therapy and the risk of Addisonian crisis associated with bilateral total adrenalectomy. We examined our large series of patients with hereditary pheochromocytoma to evaluate postsurgical outcomes, particularly in those who underwent cortical-sparing adrenalectomy.
  
  
  METHODS
  We retrospectively reviewed the outcomes of all patients with histopathologic diagnoses of hereditary pheochromocytoma treated at our institution from 1962 to 2003. Familial disease was initially determined by pedigree analysis, genetic testing, or both for multiple endocrine neoplasia (MEN) types 1, 2A, or 2B; von Hippel-Lindau disease (VHL); neurofibromatosis type 1 (NF-1); or familial paraganglioma syndrome (FP).
  
  
  RESULTS
  Adrenal pheochromocytomas were present in 56 of 59 patients (95%): MEN2A (39), MEN2B (7), VHL (6), MEN1 (2), NF-1 (2). Paragangliomas (extraadrenal pheochromocytomas) were present in the remaining 3 of 59 patients (5%): FP (2) and NF-1 (1). Thirty-eight of 56 patients with pheochromocytomas had cumulative operations resulting in total or subtotal bilateral adrenalectomy. Acute adrenal insufficiency (Addisonian crisis) occurred in 4 of these 38 patients (11%). Cortical-sparing adrenalectomy was performed in 26 patients who underwent bilateral adrenal resection; 17 (65%) were corticosteroid independent at a median followup of 71 months. Recurrent pheochromocytoma developed in an adrenal remnant in 3 of 30 patients (10%) who underwent unilateral or bilateral cortical-sparing procedures. Metastatic disease did not develop in any patient with pheochromocytoma, but has occurred in two of three patients with paragangliomas.
  
  
  CONCLUSIONS
  Our data suggest that a cortical-sparing adrenalectomy can successfully avoid the need for corticosteroid replacement in the majority of patients who undergo a bilateral adrenalectomy. Long-term followup should include monitoring of the remnant gland for recurrent pheochromocytoma with yearly biochemical screening studies.
authors.affiliation: Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA.
authors.forename: Linwah
authors.initials: L
authors.lastname: Yip
authors.forename: Jeffrey E
authors.initials: JE
authors.lastname: Lee
authors.forename: Suzanne E
authors.initials: SE
authors.lastname: Shapiro
authors.forename: Steven G
authors.initials: SG
authors.lastname: Waguespack
authors.forename: Steven I
authors.initials: SI
authors.lastname: Sherman
authors.forename: Ana O
authors.initials: AO
authors.lastname: Hoff
authors.forename: Robert F
authors.initials: RF
authors.lastname: Gagel
authors.forename: James F
authors.initials: JF
authors.lastname: Arens
authors.forename: Douglas B
authors.initials: DB
authors.lastname: Evans
mesh_terms: D000224:Addison Disease / Q000209:etiology / Q000517:prevention & control; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000235:genetics* / Q000601:surgery*; D000315:Adrenalectomy / Q000009:adverse effects / Q000379:methods*; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000150:complications; D009456:Neurofibromatosis 1 / Q000150:complications; D010235:Paraganglioma / Q000150:complications; D010673:Pheochromocytoma / Q000235:genetics* / Q000601:surgery*; D012186:Retroperitoneal Neoplasms / Q000235:genetics / Q000601:surgery; D012189:Retrospective Studies; D016896:Treatment Outcome; D001749:Urinary Bladder Neoplasms / Q000235:genetics / Q000601:surgery; D006623:von Hippel-Lindau Disease / Q000150:complications