title: Surgical resection of synchronously metastatic adrenocortical cancer.
abstract: BACKGROUND
  Metastatic adrenocortical carcinoma (ACC) is rapidly fatal, with few options for treatment. Patients with metachronous recurrence may benefit from surgical resection. The survival benefit in patients with hematogenous metastasis at initial presentation is unknown.
  
  
  METHODS
  A review of all patients undergoing surgery (European Network for the Study of Adrenal Tumors) stage IV ACC between January 2000 and December 2012 from two referral centers was performed. Kaplan-Meier estimates were analyzed for disease-free and overall survival (OS).
  
  
  RESULTS
  We identified 27 patients undergoing surgery for stage IV ACC. Metastases were present in the lung (19), liver (11), and brain (1). A complete resection (R0) was achieved in 11 patients. The median OS was improved in patients undergoing R0 versus R2 resection (860 vs. 390 days; p = 0.02). The 1- and 2-year OS was also improved in patients undergoing R0 versus R2 resection (69.9 %, 46.9 % vs. 53.0 %, 22.1 %; p = 0.02). Patients undergoing neoadjuvant therapy (eight patients) had a trend towards improved survival at 1, 2, and 5 years versus no neoadjuvant therapy (18 patients) [83.3 %, 62.5 %, 41.7 % vs. 56.8 %, 26.6 %, 8.9 %; p = 0.1]. Adjuvant therapy was associated with improved recurrence-free survival at 6 months and 1 year (67 %, 33 % vs. 40 %, 20 %; p = 0.04) but not improved OS (p = 0.63). Sex (p = 0.13), age (p = 0.95), and location of metastasis (lung, p = 0.51; liver, p = 0.67) did not correlate with OS after operative intervention. Symptoms of hormonal excess improved in 86 % of patients.
  
  
  CONCLUSIONS
  Operative intervention, especially when an R0 resection can be achieved, following systemic therapy may improve outcomes, including OS, in select patients with stage IV ACC. Response to neoadjuvant chemotherapy may be of use in defining which patients may benefit from surgical intervention. Adjuvant therapy was associated with decreased recurrence but did not improve OS.
authors.affiliation: Department of Surgery, Mayo Clinic, Rochester, MN, USA, benzondymd@gmail.com.
authors.forename: Benzon M
authors.initials: BM
authors.lastname: Dy
authors.forename: Veljko
authors.initials: V
authors.lastname: Strajina
authors.forename: Ashley K
authors.initials: AK
authors.lastname: Cayo
authors.forename: Melanie L
authors.initials: ML
authors.lastname: Richards
authors.forename: David R
authors.initials: DR
authors.lastname: Farley
authors.forename: Clive S
authors.initials: CS
authors.lastname: Grant
authors.forename: William S
authors.initials: WS
authors.lastname: Harmsen
authors.forename: Doug B
authors.initials: DB
authors.lastname: Evans
authors.forename: Elizabeth G
authors.initials: EG
authors.lastname: Grubbs
authors.forename: Keith C
authors.initials: KC
authors.lastname: Bible
authors.forename: William F
authors.initials: WF
authors.lastname: Young
authors.forename: Nancy D
authors.initials: ND
authors.lastname: Perrier
authors.forename: Florencia G
authors.initials: FG
authors.lastname: Que
authors.forename: David M
authors.initials: DM
authors.lastname: Nagorney
authors.forename: Jeffrey E
authors.initials: JE
authors.lastname: Lee
authors.forename: Geoffrey B
authors.initials: GB
authors.lastname: Thompson
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D018268:Adrenocortical Carcinoma / Q000401:mortality / Q000556:secondary / Q000601:surgery*; D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D009367:Neoplasm Staging; D009378:Neoplasms, Multiple Primary / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult