Duodenal, ampullary, and pancreatic neuroendocrine tumors: Oncologic outcomes are driven by tumor biology and tissue of origin

Abstract

BackgroundPeriampullary neuroendocrine tumors (NETs) arise from the duodenum, ampulla, and periampullary pancreas. Duodenal and ampullary NETs are rare and may have distinct biologic behavior from pancreatic NETs (P‐NETs). We examined the outcomes of these entities.MethodsAn institutional database was queried for patients undergoing resection for pancreatic head, duodenal, or ampullary NETs from 2000 to 2018. Patients with MEN1 syndrome or follow up less than 12 months were excluded.ResultsThree hundred and ten patients were identified. Tumor locations were ampulla (n = 15), duodenum (n = 35) and pancreas (n = 260). Median follow‐up and recurrence‐free survival (RFS) were 60.9 (interquartile range [IQR]: 34.8–99.3) and 171.7 (IQR: 84.0–NR) months. Clinicopathologic data and survival outcomes were similar for duodenal and ampullary NETs (RFS: p = .347 and overall survival [OS]: p = .246) and were combined into an intestinal subtype (IS) group. There were no differences in OS or RFS when comparing IS‐NET and P‐NET. On multivariate analysis, tissue of origin was not associated with risk of recurrence. The current American Joint Committee on Cancer staging guidelines, which account for origin tissue, were predictive of outcomes for all subtypes.ConclusionTissue of origin does not appear to impact long‐term outcomes when comparing IS‐NETs and P‐NETs. The AJCC staging system offers good discriminatory capacity in the context of the tissue type.

Ampullary Neuroendocrine Neoplasms: Identification of Prognostic Factors in a Multicentric Series of 119 Cases

“…These neoplasms show clinical, histologic, and immunohistochemical distinctive features in comparison with non-ampullary duodenal NENs (non-Amp-Duo-NENs) [1,2]. Indeed, Amp-NENs are larger and more commonly present with abdominal pain or jaundice due to bile duct obstruction [3,4]. Importantly, they are more frequently associated with neurofibromatosis type 1 (NF1) and tend to occur in younger individuals [4].…”

“…Indeed, Amp-NENs are larger and more commonly present with abdominal pain or jaundice due to bile duct obstruction [3,4]. Importantly, they are more frequently associated with neurofibromatosis type 1 (NF1) and tend to occur in younger individuals [4]. Although most studies showed a lack of significant prognostic differences between Amp-NENs and non-Amp-Duo-NENs, especially when only well-differentiated neuroendocrine tumors (NETs) were considered [3][4][5][6][7][8][9][10], Amp-NENs were shown to display a more aggressive behavior with a trend towards reduced overall survival in some studies [11][12][13][14][15].…”

Extended surgical resection for nonfunctioning duodenal neuroendocrine tumor

“…Endoscopic resection is recommended for lesions measuring <2 cm [ 12 ]. Tissue of origin does not seem to influence patient’s prognosis, which largely depends on tumor staging and grading [ 13–15 ]. In the patient we treated, the presence of nonfunctional NET with liver metastatic disease and a Ki67 Index >90% led us to suggest early adjuvant treatment with systemic chemotherapy and a close follow-up regimen.…”

Ampullary Large-Cell Neuroendocrine Carcinoma, a Diagnostic Challenge of a Rare Aggressive Neoplasm: A Case Report and Literature Review

“…Ampullary NENs are even more uncommonly reported, accounting for less than 0.3% of all gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) [ 5 , 6 ]. Therefore, available data are based on few case series and retrospective reviews, and ampullary neuroendocrine neoplasms are often discussed together due to the anatomic proximity with those arising from the duodenum or the pancreas, which are more often described [ 7 , 8 ].…”