title: Systematic Review and Case Series Report of Acinar Cell Carcinoma of the Pancreas.
abstract: BACKGROUND
  Acinar cell carcinoma of the pancreas is a rare malignancy representing less than 1% of all pancreatic malignancies.
  
  
  METHODS
  We report on a case series of 21 patients with acinar cell carcinoma of the pancreas treated at a high-volume quaternary center. A systematic review of the medical literature was performed that described typical therapeutic management approaches for acinar cell carcinoma of the pancreas and reported on disease control and survival rates. Data for the case series were obtained from a prospective database.
  
  
  RESULTS
  In our systematic review of 6 articles, study patients had a median age of 61 years, 66% were male, 52% had stage I/II disease, and 55% of lesions were located in the pancreatic head. The rates of median survival were approximately 47 months after resection with adjuvant therapy, 38 months for nonmetastatic, locally unresectable disease, and 17 months for metastatic disease treated with chemotherapy. Combination fluoropyrimidine-based chemotherapy regimens had better rates of disease control than other therapies. Our case series included 21 study patients, 14 of whom required resection and 7 who had metastatic disease. The rates of median survival were 40.2 ± 31.9 months in those who underwent surgery and were treated with adjuvant therapy and 13.8 ± 11.3 months for patients with metastatic disease.
  
  
  CONCLUSIONS
  Multidisciplinary treatment for acinar cell carcinoma of the pancreas should be considered due to the rarity of the disease and its lack of high-level therapeutic data. Progress in the molecular analysis of this tumor may improve outcomes through the use of personalized therapy based on underlying tumor mutations.
authors.affiliation: Department of Gastrointestinal Oncology, Moffitt Cancer Center, Tampa, FL, USA. Mokenge.Malafa@Moffitt.org.
authors.forename: Evan S
authors.initials: ES
authors.lastname: Glazer
authors.forename: Kevin G
authors.initials: KG
authors.lastname: Neill
authors.forename: Jessica M
authors.initials: JM
authors.lastname: Frakes
authors.forename: Domenico
authors.initials: D
authors.lastname: Coppola
authors.forename: Pamela J
authors.initials: PJ
authors.lastname: Hodul
authors.forename: Sara E
authors.initials: SE
authors.lastname: Hoffe
authors.forename: Jose M
authors.initials: JM
authors.lastname: Pimiento
authors.forename: Gregory M
authors.initials: GM
authors.lastname: Springett
authors.forename: Mokenge P
authors.initials: MP
authors.lastname: Malafa
mesh_terms: D018267:Carcinoma, Acinar Cell / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000473:pathology