title: Actual 10-year survivors following resection of adrenocortical carcinoma.
abstract: BACKGROUND
  Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with limited therapeutic options beyond surgical resection. The characteristics of actual long-term survivors following surgical resection for ACC have not been previously reported.
  
  
  METHODS
  Patients who underwent resection for ACC at one of 13 academic institutions participating in the US Adrenocortical Carcinoma Group from 1993 to 2014 were analyzed. Patients were stratified into four groups: early mortality (died within 2 years), late mortality (died within 2-5 years), actual 5-year survivor (survived at least 5 years), and actual 10-year survivor (survived at least 10 years). Patients with less than 5 years of follow-up were excluded.
  
  
  RESULTS
  Among the 180 patients available for analysis, there were 49 actual 5-year survivors (27%) and 12 actual 10-year survivors (7%). Patients who experienced early mortality had higher rates of cortisol-secreting tumors, nodal metastasis, synchronous distant metastasis, and R1 or R2 resections (all P < 0.05). The need for multi-visceral resection, perioperative blood transfusion, and adjuvant therapy correlated with early mortality. However, nodal involvement, distant metastasis, and R1 resection did not preclude patients from becoming actual 10-year survivors. Ten of twelve actual 10-year survivors were women, and of the seven 10-year survivors who experienced disease recurrence, five had undergone repeat surgery to resect the recurrence.
  
  
  CONCLUSIONS
  Surgery for ACC can offer a 1 in 4 chance of actual 5-year survival and a 1 in 15 chance of actual 10-year survival. Long-term survival was often achieved with repeat resection for local or distant recurrence, further underscoring the important role of surgery in managing patients with ACC. J. Surg. Oncol. 2016;114:971-976. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Surgery, Stanford University School of Medicine, Stanford, California.
authors.forename: Thuy B
authors.initials: TB
authors.lastname: Tran
authors.affiliation: Department of Surgery, Emory University, Atlanta, Georgia.
authors.forename: Lauren M
authors.initials: LM
authors.lastname: Postlewait
authors.affiliation: Department of Surgery, Emory University, Atlanta, Georgia.
authors.forename: Shishir K
authors.initials: SK
authors.lastname: Maithel
authors.affiliation: Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Jason D
authors.initials: JD
authors.lastname: Prescott
authors.affiliation: Department of Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin.
authors.forename: Tracy S
authors.initials: TS
authors.lastname: Wang
authors.affiliation: Department of Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin.
authors.forename: Jason
authors.initials: J
authors.lastname: Glenn
authors.affiliation: Department of Surgery, Ohio State University, Columbus, Ohio.
authors.forename: John E
authors.initials: JE
authors.lastname: Phay
authors.affiliation: Department of Surgery, Ohio State University, Columbus, Ohio.
authors.forename: Kara
authors.initials: K
authors.lastname: Keplinger
authors.affiliation: Department of Surgery, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Ryan C
authors.initials: RC
authors.lastname: Fields
authors.affiliation: Department of Surgery, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Linda X
authors.initials: LX
authors.lastname: Jin
authors.affiliation: Department of Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.
authors.forename: Sharon M
authors.initials: SM
authors.lastname: Weber
authors.affiliation: Department of Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.
authors.forename: Ahmed
authors.initials: A
authors.lastname: Salem
authors.affiliation: Department of Surgery, University of California San Diego, San Diego, California.
authors.forename: Jason K
authors.initials: JK
authors.lastname: Sicklick
authors.affiliation: Department of Surgery, University of California San Diego, San Diego, California.
authors.forename: Shady
authors.initials: S
authors.lastname: Gad
authors.affiliation: Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas.
authors.forename: Adam C
authors.initials: AC
authors.lastname: Yopp
authors.affiliation: Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas.
authors.forename: John C
authors.initials: JC
authors.lastname: Mansour
authors.affiliation: Department of Surgery, University of California San Francisco, San Francisco, California.
authors.forename: Quan-Yang
authors.initials: QY
authors.lastname: Duh
authors.affiliation: Department of Surgery, University of California San Francisco, San Francisco, California.
authors.forename: Natalie
authors.initials: N
authors.lastname: Seiser
authors.affiliation: Department of Surgery, Vanderbilt University, Nashville, Tennessee.
authors.forename: Carmen C
authors.initials: CC
authors.lastname: Solorzano
authors.affiliation: Department of Surgery, Vanderbilt University, Nashville, Tennessee.
authors.forename: Colleen M
authors.initials: CM
authors.lastname: Kiernan
authors.affiliation: Department of Surgery, Wake Forest Baptist Medical Center, Winston Salem, North Carolina.
authors.forename: Konstantinos I
authors.initials: KI
authors.lastname: Votanopoulos
authors.affiliation: Department of Surgery, Wake Forest Baptist Medical Center, Winston Salem, North Carolina.
authors.forename: Edward A
authors.initials: EA
authors.lastname: Levine
authors.affiliation: Department of Surgery, New York University School of Medicine, New York, New York.
authors.forename: Ioannis
authors.initials: I
authors.lastname: Hatzaras
authors.affiliation: Department of Surgery, New York University School of Medicine, New York, New York.
authors.forename: Rivfka
authors.initials: R
authors.lastname: Shenoy
authors.affiliation: Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.|Department of Surgery, Ohio State University, Columbus, Ohio.
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Pawlik
authors.affiliation: Department of Surgery, Stanford University School of Medicine, Stanford, California.
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Norton
authors.affiliation: Department of Surgery, Stanford University School of Medicine, Stanford, California.
authors.forename: George A
authors.initials: GA
authors.lastname: Poultsides
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000401:mortality* / Q000601:surgery*; D018268:Adrenocortical Carcinoma / Q000401:mortality* / Q000601:surgery*; D000328:Adult; D000368:Aged; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D016019:Survival Analysis; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology
keywords: adrenal cancer; adrenocortical carcinoma; surgery; survival