title: Clinical characteristics and outcomes from an institutional series of acinar cell carcinoma of the pancreas and related tumors.
abstract: OBJECTIVE
  Acinar cell carcinoma is a rare tumor of the exocrine pancreas. Clinical features such as prognostic information, survival, and treatment outcomes are unknown. We present the largest retrospective review to date.
  
  
  METHODS
  Thirty-nine patients with pathologically confirmed acinar neoplasms of the pancreas were identified between August 1981 and January 2001. Demographic data, tumor characteristics, and treatment information were obtained by chart review. Survival probabilities were estimated by using the Kaplan-Meier method and compared using the log-rank test.
  
  
  RESULTS
  The median survival for all patients was 19 months. On the basis of a univariate analysis, the patients' stage of disease correlated significantly with survival. The median survival of patients with localized disease was 38 months, versus 14 months for those presenting with metastases (P = 0.03). Patients who could be treated with surgery as first-line therapy had a longer survival time (36 months) compared with those who did not have surgery (14 months). Two of 18 patients who received chemotherapy and three of eight patients who received radiation had a major response.
  
  
  CONCLUSIONS
  The survival curves suggest a more aggressive cancer than pancreatic endocrine neoplasms but one that is less aggressive than ductal adenocarcinoma of the pancreas. Those patients who present with localized disease have a much better prognosis than those who present with metastases. There is a high recurrence rate after complete surgical resection, suggesting that micrometastases are present even in localized disease and that adjuvant therapies may be indicated. Chemotherapy and radiation afford disappointing results, however, and novel therapies are needed.
authors.affiliation: Memorial Sloan-Kettering Cancer Center, Gastrointestinal Oncology Service, Department of Medicine, New York, NY 10021, USA.
authors.forename: Kyle D
authors.initials: KD
authors.lastname: Holen
authors.forename: David S
authors.initials: DS
authors.lastname: Klimstra
authors.forename: Amanda
authors.initials: A
authors.lastname: Hummer
authors.forename: Mithat
authors.initials: M
authors.lastname: Gonen
authors.forename: Kevin
authors.initials: K
authors.lastname: Conlon
authors.forename: Murray
authors.initials: M
authors.lastname: Brennan
authors.forename: Leonard B
authors.initials: LB
authors.lastname: Saltz
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018267:Carcinoma, Acinar Cell / Q000401:mortality / Q000628:therapy*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D010190:Pancreatic Neoplasms / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome