title: Aggressive approach to acinar cell carcinoma of the pancreas: a single-institution experience and a literature review.
abstract: OBJECTIVE
  Acinar cell carcinomas (ACCs) are a rare pancreatic tumor group with no standardized treatment. The aim of the study is to analyze the clinical and pathologic characteristics of our series and to review the current literature.
  
  
  METHODS
  Retrospective review of prospectively collected data from 1990 to 2007 included patients who underwent pancreatic resection for histologically proven ACCs. All specimens of ACC were rereviewed by an expert pathologist. Follow-up was updated to October 2009. A literature search was performed by Pubmed and COCHRANE library.
  
  
  RESULTS
  Among 1,210 patients who underwent pancreatic resection, we identified nine ACCs. R0 resection was possible for all but one R1. We had no major complications and no mortality. All nine cases were diagnosed as pure ACCs. Five patients received adjuvant chemotherapy. Median overall survival was 31 months, while median disease-free survival was 18 months. All patients developed liver metastases, requiring modification of chemotherapeutic schema, radiofrequency ablation techniques, or reiterate surgery. Currently, only one patient is alive without evidence of disease 85 months after pancreatic resection. One patient is alive 52 months after operation, with evidence of recurrent disease.
  
  
  CONCLUSIONS
  ACC represents a rare solid tumor of the pancreas. Prognosis is dismal, although, compared to the more common ductal adenocarcinoma, survival appears to be longer. Patients with metastatic disease might benefit from aggressive multimodality treatments.
authors.affiliation: Surgical Department, Verona University Hospital, Piazzale La Scuro 10, Verona, Italy. giovanni.butturini@ospedaleuniverona.it
authors.forename: Giovanni
authors.initials: G
authors.lastname: Butturini
authors.forename: Michele
authors.initials: M
authors.lastname: Pisano
authors.forename: Aldo
authors.initials: A
authors.lastname: Scarpa
authors.forename: Mirko
authors.initials: M
authors.lastname: D'Onofrio
authors.forename: Alessandra
authors.initials: A
authors.lastname: Auriemma
authors.forename: Claudio
authors.initials: C
authors.lastname: Bassi
mesh_terms: D000328:Adult; D000368:Aged; D001707:Biopsy, Needle; D018267:Carcinoma, Acinar Cell / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D017024:Chemotherapy, Adjuvant; D015331:Cohort Studies; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D055088:Early Detection of Cancer; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D053208:Kaplan-Meier Estimate; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010180:Pancreatectomy / Q000379:methods; D010190:Pancreatic Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D018714:Radiotherapy, Adjuvant; D035583:Rare Diseases; D012189:Retrospective Studies; D016019:Survival Analysis; D013997:Time Factors; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome
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