title: Mayo Clinic experience with very rare exocrine pancreatic neoplasms.
abstract: OBJECTIVE
  Limited data are available to guide the management of very rare exocrine neoplasms of the pancreas (VREP). Available evidence suggests that VREP have different risk factors and prognoses from those of adenocarcinoma of the pancreas. The primary objectives for this study were to determine the survival, comorbidities, and response to treatment of patients seen at Mayo Clinic with VREP.
  
  
  METHODS
  We reviewed patients from 1975 to 2005 who had VREP and compared them to patients with adenocarcinomas that were matched for TNM, grade, and decade of treatment.
  
  
  RESULTS
  Sixty-six patients with VREP were identified. The most commonly identified neoplasms were acinar cell carcinoma (n = 15), small cell carcinoma (n = 12), and squamous cell carcinoma (n = 8). Abdominal discomfort and jaundice were the most common presenting symptoms. The median overall survival for patients with VREP, 10.4 months (range, 3.7-23 months), was better than that for matched controls, 8.2 months (range, 4-15.4 months) (P = 0.01). There was no difference in the survival of patients with stage 4 disease between cases, 8 months (range, 2.3-21.8 months), and controls, 6.7 months (range, 2.3-10.8 months) (P = 0.17).
  
  
  CONCLUSIONS
  We present one of the largest series of VREP to date. The overall survival of all patients with VREP was better than matched controls, but no statistical difference was seen between the groups with stage 4 disease.
authors.affiliation: Department of Oncology, Mayo Clinic, Rochester, MN 55905, USA.
authors.forename: Aaron
authors.initials: A
authors.lastname: Mansfield
authors.forename: Alfonso
authors.initials: A
authors.lastname: Tafur
authors.forename: Patrick
authors.initials: P
authors.lastname: Smithedajkul
authors.forename: Michele
authors.initials: M
authors.lastname: Corsini
authors.forename: Fernando
authors.initials: F
authors.lastname: Quevedo
authors.forename: Robert
authors.initials: R
authors.lastname: Miller
mesh_terms: D000328:Adult; D000368:Aged; D018267:Carcinoma, Acinar Cell / Q000401:mortality; D018288:Carcinoma, Small Cell / Q000401:mortality; D002294:Carcinoma, Squamous Cell / Q000401:mortality; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010190:Pancreatic Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D035583:Rare Diseases / Q000401:mortality* / Q000473:pathology / Q000628:therapy*