Journal of Gastrointestinal Surgery

, Volume 14, Issue 3, pp 541–548

Non-functional Neuroendocrine Carcinoma of the Pancreas: Incidence, Tumor Biology, and Outcomes in 2,158 Patients

  • Jan Franko
  • Wentao Feng
  • Linwah Yip
  • Elizabeth Genovese
  • A. James Moser
Original Article

DOI: 10.1007/s11605-009-1115-0

Cite this article as:
Franko, J., Feng, W., Yip, L. et al. J Gastrointest Surg (2010) 14: 541. doi:10.1007/s11605-009-1115-0

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).

Conclusion

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.

Keywords

Pancreatic endocrine tumorIslet cell carcinomaCarcinoidIncidenceMetastasectomyEnucleation

Copyright information

© The Society for Surgery of the Alimentary Tract 2009

Authors and Affiliations

  • Jan Franko
    • 1
  • Wentao Feng
    • 2
  • Linwah Yip
    • 1
  • Elizabeth Genovese
    • 1
  • A. James Moser
    • 1
    • 3
  1. 1.Division of Surgical OncologyUniversity of Pittsburgh Medical CenterPittsburghUSA
  2. 2.Department of BiostatisticsUniversity of PittsburghPittsburghUSA
  3. 3.Department of SurgeryUniversity of Pittsburgh Medical CenterPittsburghUSA