title: Non-functional neuroendocrine carcinoma of the pancreas: incidence, tumor biology, and outcomes in 2,158 patients.
abstract: OBJECTIVE
  Pancreatic neuroendocrine cancer is a rare, indolent malignancy with no effective systemic therapy currently available. This population-based analysis evaluated the hypothesis that long-term survival benefit is greater with aggressive, rather than limited, surgical therapy.
  
  
  METHODS
  Non-functional pancreatic neuroendocrine carcinoma (NF-pNEC) cases diagnosed from 1973 to 2004 were retrieved from the SEER database.
  
  
  RESULTS
  A total of 2,158 patients with NF-pNEC were identified, representing 2% of all pancreatic malignancies. The annual incidence increased from 1.4 to 3.0 per million during the study period. On average, tumors measured 59 +/- 35 mm (median 50), and age at diagnosis was 59 +/- 15 years; 29% of patients were younger than 50. Nodal (44%) and systemic metastases (60%) were common. Overall the 5-, 10-, and 20-year survival rates were 33%, 17%, and 10%, respectively. Removal of the primary tumor significantly prolonged survival in the entire cohort (median 1.2 vs. 8.4 years; p < 0.001) and among those with metastases (median 1.0 vs. 4.8 years; p < 0.001). No survival difference was seen between enucleation and resection of the primary tumor (median 10.2 versus 9.2 years, p = 0.456).
  
  
  CONCLUSIONS
  This study suggests that surgical therapy improves survival among patients with localized, as well as metastatic, NF-pNEC. Enucleation may be oncologically equivalent to resection.
authors.affiliation: Division of Surgical Oncology, University of Pittsburgh Medical Center, Pittsburgh, PA 15261, USA.
authors.forename: Jan
authors.initials: J
authors.lastname: Franko
authors.forename: Wentao
authors.initials: W
authors.lastname: Feng
authors.forename: Linwah
authors.initials: L
authors.lastname: Yip
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Genovese
authors.forename: A James
authors.initials: AJ
authors.lastname: Moser
mesh_terms: D000328:Adult; D017677:Age Distribution; D000368:Aged; D001707:Biopsy, Needle; D018278:Carcinoma, Neuroendocrine / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology* / Q000556:secondary / Q000601:surgery; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009361:Neoplasm Invasiveness / Q000473:pathology*; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology; D009367:Neoplasm Staging; D010180:Pancreatectomy / Q000379:methods; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology* / Q000601:surgery; D011336:Probability; D011379:Prognosis; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D018426:SEER Program; D017678:Sex Distribution; D016019:Survival Analysis