Background:Functional pancreatic neuroendocrine tumors(f-PanNETs)are rare tumors of the pancreas that are associated with excess hormone production.A majority of literature available on these tumors is limited to case...Background:Functional pancreatic neuroendocrine tumors(f-PanNETs)are rare tumors of the pancreas that are associated with excess hormone production.A majority of literature available on these tumors is limited to case reports and small series and discusses a particular subtype.Here,we report the clinicopathological features and outcomes of all f-PanNETs from a high-volume pancreatectomy center.Methods:A prospectively maintained database was used to identify patients who underwent resection for f-PanNETs between January 1995 and December 2015.Patients were stratified by the type of f-PanNET,and their clinicopathological features and outcomes were analyzed and reported.Results:We identified 69 patients with a mean age of 52±16 years,and the majority were female(62%).The most common type of f-PanNET were insulinomas(70%)followed by gastrinomas(15%),and vasoactive intestinal peptidomas(VIPomas)(10%).There was considerable heterogeneity in the presenting symptoms.Patients with insulinomas presented with smaller tumors compared to the other subtypes.Nodal disease was most frequent in patients with VIPomas(71%),and gastrinomas(50%).The median overall survival(OS)was 7.4 years.Elevated Ki-67 and age>65 years were associated with poorer OS.Recurrence was observed in 8 patients(12%)and was most frequent in patients with VIPomas.Recurrence was associated with a poorer OS(P=.012).Conclusion:f-PanNETs are exceedingly rare and present with a wide variety of symptoms,histopathological findings,and outcomes.Surgical resection can help achieve good long-term survival.展开更多
文摘Background:Functional pancreatic neuroendocrine tumors(f-PanNETs)are rare tumors of the pancreas that are associated with excess hormone production.A majority of literature available on these tumors is limited to case reports and small series and discusses a particular subtype.Here,we report the clinicopathological features and outcomes of all f-PanNETs from a high-volume pancreatectomy center.Methods:A prospectively maintained database was used to identify patients who underwent resection for f-PanNETs between January 1995 and December 2015.Patients were stratified by the type of f-PanNET,and their clinicopathological features and outcomes were analyzed and reported.Results:We identified 69 patients with a mean age of 52±16 years,and the majority were female(62%).The most common type of f-PanNET were insulinomas(70%)followed by gastrinomas(15%),and vasoactive intestinal peptidomas(VIPomas)(10%).There was considerable heterogeneity in the presenting symptoms.Patients with insulinomas presented with smaller tumors compared to the other subtypes.Nodal disease was most frequent in patients with VIPomas(71%),and gastrinomas(50%).The median overall survival(OS)was 7.4 years.Elevated Ki-67 and age>65 years were associated with poorer OS.Recurrence was observed in 8 patients(12%)and was most frequent in patients with VIPomas.Recurrence was associated with a poorer OS(P=.012).Conclusion:f-PanNETs are exceedingly rare and present with a wide variety of symptoms,histopathological findings,and outcomes.Surgical resection can help achieve good long-term survival.
