title: The role of surgery in the management of recurrent adrenocortical carcinoma.
abstract: BACKGROUND
  Surgery is the standard of care for localized adrenocortical carcinomas, but its role for recurrent disease is not well defined.
  
  
  OBJECTIVE
  Our objective was to evaluate clinical outcome after surgery for recurrence.
  
  
  METHODS
  We conducted a retrospective analysis in 154 patients with first recurrence after initial radical resection from the German Adrenocortical Carcinoma Registry.
  
  
  METHODS
  We evaluated progression-free survival (PFS) and overall survival (OS) by Kaplan-Meier method and identified prognostic factors by Cox regression analysis.
  
  
  RESULTS
  A total of 101 patients underwent repeated surgery (radical resection, n = 78), and 99 received (additional) nonsurgical therapy. After a median of 6 (1-221) months, 144 patients (94%) experienced progression. Multivariate analysis adjusted for age, sex, tumor burden, time to first recurrence (TTFR), surgery for recurrence (including resection status), and additional therapy indicated that only two factors were significantly associated with shorter PFS [hazard ratio for progression: for TTFR ≤ 12 months, 1.8 (95% confidence interval = 1.3-2.6) vs. TTFR > 12 months; for macroscopically incomplete resection, 3.4 (1.5-7.9), and for no surgery, 3.4 (1.6-7.0) vs. microscopically complete (R0)-resection and OS [hazard ratio for death: for TTFR > 12 months, 3.1 (2.0-4.7) vs. TTFR ≤ 12 months; for macroscopically incomplete resection, 2.7 (1.1-6.9), and no surgery, 4.2 (1.8-9.6) vs. R0-resection]. Patients who had both TTFR over 12 months and R0-resection of recurrent tumors (n = 22) had the best prognosis (median PFS, 24 months; median OS, >60 months).
  
  
  CONCLUSIONS
  The best predictors of prolonged survival after first recurrence are TTFR over 12 months and R0-resection. Our data suggest that patients with longer TTFR and tumors amenable to radical resection should be operated, whereas individualized treatment decisions are needed for patients with short TTFR or with not completely resectable tumors.
authors.affiliation: Department of Internal Medicine I, Endocrine and Diabetes Unit, University Hospital Würzburg, University of Würzburg, Oberdürrbacher Strasse 6, D-97080 Würzburg, Germany.
authors.forename: Ilknur
authors.initials: I
authors.lastname: Erdogan
authors.forename: Timo
authors.initials: T
authors.lastname: Deutschbein
authors.forename: Christian
authors.initials: C
authors.lastname: Jurowich
authors.forename: Matthias
authors.initials: M
authors.lastname: Kroiss
authors.forename: Christina
authors.initials: C
authors.lastname: Ronchi
authors.forename: Marcus
authors.initials: M
authors.lastname: Quinkler
authors.forename: Jens
authors.initials: J
authors.lastname: Waldmann
authors.forename: Holger S
authors.initials: HS
authors.lastname: Willenberg
authors.forename: Felix
authors.initials: F
authors.lastname: Beuschlein
authors.forename: Christian
authors.initials: C
authors.lastname: Fottner
authors.forename: Silke
authors.initials: S
authors.lastname: Klose
authors.forename: Anke
authors.initials: A
authors.lastname: Heidemeier
authors.forename: David
authors.initials: D
authors.lastname: Brix
authors.forename: Wiebke
authors.initials: W
authors.lastname: Fenske
authors.forename: Stefanie
authors.initials: S
authors.lastname: Hahner
authors.forename: Joachim
authors.initials: J
authors.lastname: Reibetanz
authors.forename: Bruno
authors.initials: B
authors.lastname: Allolio
authors.forename: Martin
authors.initials: M
authors.lastname: Fassnacht
mesh_terms: D000293:Adolescent; D000306:Adrenal Cortex Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery* / Q000628:therapy*; D000315:Adrenalectomy / Q000379:methods / Q000706:statistics & numerical data*; D018268:Adrenocortical Carcinoma / Q000401:mortality / Q000473:pathology / Q000601:surgery* / Q000628:therapy*; D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012042:Registries; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult