NESS - The National Rise of Primary Pancreatic Carcinoid Tumors: Comparison to Functional and Non-functional Pancreatic Neuroendocrine Tumors (PNETs)

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The National Rise of Primary Pancreatic Carcinoid Tumors: Comparison to Functional and Non-functional Pancreatic Neuroendocrine Tumors (PNETs)
*Gyulnara G Kasumova, *Mariam F Eskander, *Omidreza Tabatabaie, *Abhishek Tadikonda, *Sing Chau Ng, Jennifer F Tseng
Beth Israel Deaconess Medical Center, Boston, MA

Objective:To compare pancreatic carcinoid tumors to other functional and non-functional pancreatic neuroendocrine tumors.
Design:Retrospective cohort study.
Setting:National Cancer Data Base 2004-2013.
Patients:Adults aged 18-90+ with primary pancreatic carcinoid, functional (insulinoma, glucagonoma, gastrinoma, somatostatinoma) and non-functional neuroendocrine tumors.
Interventions: None
Main Outcome Measures:5-year overall survival and hazard ratios.
Results:Of 10,752 patients, 84.7% were diagnosed with non-functional PNET and 12.7% with carcinoid. While the number of functional PNET has remained relatively constant, the numbers of non-functional and carcinoid tumors rose dramatically - 36 carcinoid tumors reported in 2004 and 497 in 2013. Most patients were male (53.0%) who had significantly more non-functional tumors (53.7%) while females had more functional tumors (52.9%) (p=0.0016). For all histologies, body/tail location was most common (42.8%). Carcinoid tumors underwent more resections than functional and non-functional tumors (68.5%, 64.5%, 54.5%, p<0.0001). Carcinoid tumors were more likely to be well-differentiated (60.6%) than functional and non-functional tumors (41.3% vs. 40.3%, p<0.0001). Overall 5-year survival was higher for carcinoid tumors (63.1%) compared to functional (58.3%) and non-functional (52.6%) tumors (logrank, p<0.0001). For resected patients, 5-year survival was further enhanced for carcinoid tumors (89.2%), but comparable for functional and non-functional (76.6% and 78.7%) (logrank, p<0.0001). On multivariate Cox regression of resected cohort, hazard ratios were significantly higher for patients with functional (HR 1.8) and non-functional (HR 1.5) tumors compared to carcinoid tumors (see figure).

Conclusions:Primary pancreatic carcinoid tumors are increasingly diagnosed and demonstrate a survival advantage over other PNETs. Resection remains the standard of care.

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