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

• Published in: Journal of gastrointestinal surgery Vol. 14; no. 3; pp. 541 - 548
• Main Authors: Franko, Jan, Feng, Wentao, Yip, Linwah, Genovese, Elizabeth, Moser, A. James
• Format: Journal Article
• Language: English
• Published: New York Springer-Verlag 01.03.2010; Springer Nature B.V
• Subjects: Adult; Age Distribution; Aged; Biology; Biopsy, Needle; Carcinoma, Neuroendocrine - diagnosis; Carcinoma, Neuroendocrine - epidemiology; Carcinoma, Neuroendocrine - pathology; Carcinoma, Neuroendocrine - secondary; Carcinoma, Neuroendocrine - surgery; Disease-Free Survival; Female; Gastroenterology; Humans; Immunohistochemistry; Incidence; Lymphatic Metastasis; Male; Medical research; Medicine; Medicine & Public Health; Middle Aged; Multivariate Analysis; Neoplasm Invasiveness - pathology; Neoplasm Recurrence, Local - mortality; Neoplasm Recurrence, Local - pathology; Neoplasm Staging; Original Article; Pancreatectomy - methods; Pancreatic Neoplasms - diagnosis; Pancreatic Neoplasms - epidemiology; Pancreatic Neoplasms - pathology; Pancreatic Neoplasms - surgery; Probability; Prognosis; Proportional Hazards Models; Registries; Retrospective Studies; Risk Assessment; SEER Program; Sex Distribution; Surgery; Survival Analysis; Enucleation; Islet cell carcinoma; Metastasectomy; Carcinoid; Pancreatic endocrine tumor; Incidence
• ISSN: 1091-255X; 1873-4626
• DOI: 10.1007/s11605-009-1115-0

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.