title: Resection of adrenocortical carcinoma liver metastasis: is it justified?
abstract: BACKGROUND
  Adrenocortical carcinoma (ACC) liver metastases (LM) represent a therapeutic challenge, and it is unclear whether resection is justified. This study assesses long-term outcome and prognostic factors after liver resection for metastatic ACC.
  
  
  METHODS
  Patients who underwent resection of ACC LM were identified from institutional databases. Recurrence, survival, and tumor characteristics, including β-catenin and TP53 status based on immunohistochemistry and sequencing, were reviewed. The prognostic value of variables was assessed with log-rank test for univariate analysis and Cox proportional hazard models for multivariate analysis.
  
  
  RESULTS
  From 1978 to 2009, 28 patients (20 females; median age, 45 years), including 11 with synchronous metastasis and 3 with extrahepatic metastasis, underwent resection for ACC LM (major hepatectomy in 61%). Postoperative mortality was nil and morbidity 55%. On pathological examination, tumors were multiple in 68%, with a median size of 43 mm, and resections were R0, 1, and 2 in 59%, 33%, and 7%, respectively. All 28 patients developed recurrent disease, which was treated surgically in 11, including repeat hepatectomy in 4. Of the 15 patients with adequate tissue for analysis, β-catenin immunostaining was positive in 7, with 4 corresponding CTNNB1 mutations associated with decreased survival; p53 staining was positive in 5 (4 with corresponding TP53 mutations). The median disease-free and overall survival after hepatectomy was 7 and 31.5 months, respectively, with a 5-year survival of 39%. In multivariate analysis, nonfunctional tumor and surgical treatment of recurrence were independent predictors of good outcome.
  
  
  CONCLUSIONS
  In selected patients with ACC LM, resection is associated with long-term survival and is, therefore, justified but rarely curative.
authors.affiliation: Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Gaujoux
authors.forename: Hikmat
authors.initials: H
authors.lastname: Al-Ahmadie
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Allen
authors.forename: Mithat
authors.initials: M
authors.lastname: Gonen
authors.forename: Jinru
authors.initials: J
authors.lastname: Shia
authors.forename: Michael
authors.initials: M
authors.lastname: D'Angelica
authors.forename: Ronald
authors.initials: R
authors.lastname: Dematteo
authors.forename: Yuman
authors.initials: Y
authors.lastname: Fong
authors.forename: Leslie
authors.initials: L
authors.lastname: Blumgart
authors.forename: William R
authors.initials: WR
authors.lastname: Jarnagin
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D018268:Adrenocortical Carcinoma / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D000328:Adult; D014408:Biomarkers, Tumor / Q000235:genetics* / Q000378:metabolism; D004273:DNA, Neoplasm / Q000235:genetics; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy / Q000401:mortality*; D006801:Humans; D007124:Immunoenzyme Techniques; D008113:Liver Neoplasms / Q000401:mortality / Q000556:secondary / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery; D009367:Neoplasm Staging; D016133:Polymerase Chain Reaction; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D016159:Tumor Suppressor Protein p53 / Q000235:genetics / Q000378:metabolism; D051153:Wnt Proteins / Q000235:genetics / Q000378:metabolism; D051176:beta Catenin / Q000235:genetics / Q000378:metabolism
chemical_list: D014408:Biomarkers, Tumor; C495270:CTNNB1 protein, human; D004273:DNA, Neoplasm; C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53; D051153:Wnt Proteins; D051176:beta Catenin