title: Surgical Treatment of Malignant Pheochromocytoma and Paraganglioma: Retrospective Case Series.
abstract: BACKGROUND
  Pheochromocytoma and paraganglioma (PPGL) are rare neoplasms; about 10% are malignant. Literature regarding possible benefit from resection is extremely limited.
  
  
  METHODS
  A 20 year review of all patients undergoing surgery for malignant PPGL at the Mayo Clinic Rochester Campus between 1994 and June 2014 was performed.
  
  
  RESULTS
  We identified 34 patients undergoing surgery for malignant PPGL. Median follow up was 6 and 5 years survival was 90% (median 11 years). Complete resection (R0) was achieved in 14 patients (41%). Median disease-free survival was 4.6 years for patients with R0 resection (up to 12 years). Only eight patients (23%) were disease-free on last follow up. Elevated preoperative fractionated metanephrines or catecholamines were documented in 23 patients (68%); these normalized in 13 of 23 patients (56%) postoperatively-with symptom relief in 15 of 18 preoperatively symptomatic patients (79%). Among 23 patients with hormone-producing tumors, significant reduction in number of antihypertensive medications was also noted postoperatively; 11 patients have remained off all antihypertensives, 6 required 1 medication, 1 required 2, while 5 required full blockade with phenoxybenzamine and a beta-adrenergic blocker.
  
  
  CONCLUSIONS
  Surgery plays a significant role in the management of selected malignant PPGL. Resection can be effective in normalizing or significantly reducing levels of catecholamines and metanephrines, and can improve hormone-related symptoms and hypertension. Surgical resection, either complete or incomplete, is associated with durable survival despite a high rate of tumor recurrence.
authors.affiliation: Department of Surgery, Mayo Clinic, Rochester, MN, USA. veljkostrajina@yahoo.com.
authors.forename: Veljko
authors.initials: V
authors.lastname: Strajina
authors.affiliation: Department of Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: Benzon M
authors.initials: BM
authors.lastname: Dy
authors.affiliation: Department of Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: David R
authors.initials: DR
authors.lastname: Farley
authors.affiliation: Department of Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: Melanie L
authors.initials: ML
authors.lastname: Richards
authors.affiliation: Department of Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: Travis J
authors.initials: TJ
authors.lastname: McKenzie
authors.affiliation: Division of Medical Oncology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Keith C
authors.initials: KC
authors.lastname: Bible
authors.affiliation: Department of Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: Florencia G
authors.initials: FG
authors.lastname: Que
authors.affiliation: Department of Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: David M
authors.initials: DM
authors.lastname: Nagorney
authors.affiliation: Division of Endocrinology, Metabolism, Nutrition and Diabetes, Mayo Clinic, Rochester, MN, USA.
authors.forename: William F
authors.initials: WF
authors.lastname: Young
authors.affiliation: Department of Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: Geoffrey B
authors.initials: GB
authors.lastname: Thompson
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000473:pathology / Q000601:surgery*; D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D010235:Paraganglioma / Q000473:pathology / Q000601:surgery*; D010673:Pheochromocytoma / Q000473:pathology / Q000601:surgery*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult