title: Acinar cell carcinoma of the pancreas: a rare disease with different diagnostic and therapeutic implications than ductal adenocarcinoma.
abstract: OBJECTIVE
  Acinar cell carcinoma (ACC) of the pancreas is a very rare cancer, constituting 1 % of all malignant non-endocrine pancreatic tumors. Only very limited data exist to guide treatment in patients with advanced ACC.
  
  
  METHODS
  Between 2000 and 2015, 15 patients with ACC were diagnosed and/or treated at our high-volume comprehensive cancer center. Medical records and correlating serum levels of the potential serum tumor markers CA 19-9, CEA and lipase were analyzed retrospectively.
  
  
  RESULTS
  A substantial antitumor activity was observed for treatment regimens containing 5-FU and oxaliplatin with partial responses or prolonged disease stabilizations (>12 months) observed in 6 out of 7 patients (86 %). Activity was also observed for single-agent 5-FU and its oral prodrugs. Serum lipase levels were elevated in 7 of 12 patients with advanced disease (58 %), whereas CEA and CA 19-9 seemed to be of minor importance for ACC (elevated pre-treatment levels in 4/12 and 3/12 cases, respectively). In selected patients, repeated serum lipase measurements were available and accurately predicted response to chemotherapy and relapse after surgery.
  
  
  CONCLUSIONS
  5-FU- and oxaliplatin-containing regimens are active in advanced ACC. Lipase kinetics may be a useful novel tool to monitor the course of disease as well as treatment effects in ACC.
authors.affiliation: Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany.
authors.forename: Stephan
authors.initials: S
authors.lastname: Kruger
authors.affiliation: Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Haas
authors.affiliation: Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany.
authors.forename: Philipp Johannes
authors.initials: PJ
authors.lastname: Burger
authors.affiliation: Institute of Pathology, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany.
authors.forename: Steffen
authors.initials: S
authors.lastname: Ormanns
authors.affiliation: Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany.
authors.forename: Dominik Paul
authors.initials: DP
authors.lastname: Modest
authors.affiliation: Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany.
authors.forename: Christoph Benedikt
authors.initials: CB
authors.lastname: Westphalen
authors.affiliation: Department of General, Visceral, Transplantation, Vascular and Thoracic Surgery, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany.
authors.forename: Axel
authors.initials: A
authors.lastname: Kleespies
authors.affiliation: Department of General, Visceral, Transplantation, Vascular and Thoracic Surgery, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany.
authors.forename: Martin Kurt
authors.initials: MK
authors.lastname: Angele
authors.affiliation: Department of General, Visceral, Transplantation, Vascular and Thoracic Surgery, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany.
authors.forename: Werner
authors.initials: W
authors.lastname: Hartwig
authors.affiliation: Department of General, Visceral and Tumor Surgery, University of Cologne, 50937, Köln-Lindenthal, Germany.
authors.forename: Christiane Josephine
authors.initials: CJ
authors.lastname: Bruns
authors.affiliation: Institute of Pathology, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Kirchner
authors.affiliation: Department of General, Visceral, Transplantation, Vascular and Thoracic Surgery, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Werner
authors.affiliation: Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany.
authors.forename: Volker
authors.initials: V
authors.lastname: Heinemann
authors.affiliation: Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany. stefan.boeck@med.uni-muenchen.de.
authors.forename: Stefan
authors.initials: S
authors.lastname: Boeck
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002166:Camptothecin / Q000031:analogs & derivatives / Q000627:therapeutic use; D018267:Carcinoma, Acinar Cell / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D021441:Carcinoma, Pancreatic Ductal / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D005260:Female; D005472:Fluorouracil / Q000627:therapeutic use; D006801:Humans; D002955:Leucovorin / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009944:Organoplatinum Compounds / Q000008:administration & dosage / Q000627:therapeutic use; D000077150:Oxaliplatin; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D015995:Prevalence; D011379:Prognosis; D035583:Rare Diseases; D012189:Retrospective Studies; D016019:Survival Analysis
chemical_list: D009944:Organoplatinum Compounds; D000077150:Oxaliplatin; D002955:Leucovorin; D005472:Fluorouracil; D002166:Camptothecin
keywords: Acinar cell carcinoma; Chemotherapy; Pancreas; Pancreatic cancer; Serum lipase; Tumor marker