Survival and Incidence Patterns of Pancreatic Neuroendocrine Tumors Over the Last 2 Decades: A SEER Database Analysis

BackgroundPancreatic neuroendocrine tumors (pNETs) are rare cancers with outcomes determined by multiple factors including grade, stage, and clinical presentation. In this study, we aimed to determine the prognosis of patients with pNETs using a large population-based database.Materials and MethodsIn this population-based study, we identified patients with pNETs from the SEER 18 registry (2000-2016) using a combination of ICD-O-3 and histology codes. We calculated age-adjusted incidence rates using SEER*Stat 8.3.5. In addition, we analyzed overall survival (OS) using the Kaplan-Meier method, and investigated prognostic factors using a multivariable Cox proportional hazards model.ResultsA total of 8944 pNETs patients were identified. Annual incidence rates increased from 0.27 to 1.00 per 100 000. This was largely explained by an increase in number of patients diagnosed with localized disease in more recent years (2012-2016). Median OS was 68 months (95% CI [64, 73]) and 5-year OS rates in localized, regional, and metastatic disease were 83%, 67%, and 28%, respectively. There was a significant improvement in OS for patients diagnosed between 2009 and 2016 (median OS 85 months) compared with those diagnosed between 2000 and 2008 (median OS 46 months) (HR 0.66; 95% CI [0.62, 0.70]). This improvement in OS was consistent across all stages.Conclusions and RelevanceThis study shows a steady increase pNETs incidence with notable stage migration to earlier stages in recent years. This increase in incidence is accompanied by a significant improvement in survival across different disease stages.     

Chylolymphatic mesenteric cyst with midgut volvulus in an adolescent: a peculiar presentation

Mesenteric cysts are rare intra-abdominal masses in the pediatric population, with the chylolymphatic variant comprising only 7.3% of all abdominal cysts. These cysts can have a varied clinical presentation, ranging from asymptomatic cystic masses to intestinal obstruction. We report a 16-year-old female, who presented with acute abdominal pain and vomiting, and was diagnosed with an intra-abdominal cystic mass with midgut volvulus accompanied by the classical “whirlpool sign” on imaging.She underwent laparoscopic abdominal exploration, which revealed a large chylolymphatic mesenteric cyst associated with feeder lymph vessels. This cyst had resulted in volvulus of the small bowel. The bowel was detorsed and found to be viable, and the cyst was removed en bloc after suction evacuation with ligation of the lymphatic feeder vessels.Midgut volvulus in the pediatric population is usually secondary to malrotation, but in this case the patient''s small intestine was not found to be malrotated, and hence we present this case and urge physicians to consider a diagnosis of midgut volvulus even in the absence of malrotation in a child with a cystic abdominal mass presenting as intestinal obstruction.