Presentation and outcome of mixed neuroendocrine non-neuroendocrine neoplasms of the pancreas
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Presentation and outcome of mixed neuroendocrine non-neuroendocrine neoplasms of the pancreas. / Nießen, Anna; Schimmack, Simon; Weber, Tim F; Mayer, Philipp; Bergmann, Frank; Hinz, Ulf; Büchler, Markus W; Strobel, Oliver.
In: PANCREATOLOGY, Vol. 21, No. 1, 01.2021, p. 224-235.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Presentation and outcome of mixed neuroendocrine non-neuroendocrine neoplasms of the pancreas
AU - Nießen, Anna
AU - Schimmack, Simon
AU - Weber, Tim F
AU - Mayer, Philipp
AU - Bergmann, Frank
AU - Hinz, Ulf
AU - Büchler, Markus W
AU - Strobel, Oliver
N1 - Copyright © 2020 IAP and EPC. Published by Elsevier B.V. All rights reserved.
PY - 2021/1
Y1 - 2021/1
N2 - BACKGROUND/OBJECTIVES: Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) of the pancreas and periampullary region are extremely rare and heterogeneous malignancies. Literature is sparse, clinical management is not standardized and little is known about survival outcomes. The aim of this study was to identify pathological and radiological features of MiNEN and assess the outcome of surgical management.METHODS: Patients undergoing surgery for pancreatic and periampullary MiNEN between 2001 and 2019 were retrospectively analysed based on a prospective database. Histological, radiological and clinical features were assessed. Survival was analysed in a nested case-control study and matched-pair analyses with pure neuroendocrine neoplasms (pNEN) and ductal adeno- or acinar cell carcinomas of the pancreas. A literature review with focus on survival after surgical resection was additionally performed.RESULTS: Of 13 patients with MiNEN, 5 had acinar-MiNEN and 8 adeno-MiNEN. Two of 5 (40%) acinar-MiNEN and one adeno-MiNEN patients had liver metastases. All but one adeno-MiNEN (88%) showed preoperative radiological features of pancreatic adenocarcinoma, 3 of 5 (60%) acinar-MiNEN exhibited mainly neuroendocrine features. No surgical mortality was observed. The 5-year overall survival rate in all MiNEN was 40%. Five-year survival rate was 58% in adeno-MiNEN and comparable to that of matched ductal adenocarcinomas (36%) and pNEN (48%). Five-year overall survival rate was 20% in acinar-MiNEN, compared to 39% in acinar carcinoma patients and 59% in matched pNEN patients.CONCLUSIONS: MiNEN are rare and difficult to distinguish from pure adenocarcinoma or neuroendocrine neoplasm preoperatively. Surgical resection would therefore be the treatment of choice in localized tumors.
AB - BACKGROUND/OBJECTIVES: Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) of the pancreas and periampullary region are extremely rare and heterogeneous malignancies. Literature is sparse, clinical management is not standardized and little is known about survival outcomes. The aim of this study was to identify pathological and radiological features of MiNEN and assess the outcome of surgical management.METHODS: Patients undergoing surgery for pancreatic and periampullary MiNEN between 2001 and 2019 were retrospectively analysed based on a prospective database. Histological, radiological and clinical features were assessed. Survival was analysed in a nested case-control study and matched-pair analyses with pure neuroendocrine neoplasms (pNEN) and ductal adeno- or acinar cell carcinomas of the pancreas. A literature review with focus on survival after surgical resection was additionally performed.RESULTS: Of 13 patients with MiNEN, 5 had acinar-MiNEN and 8 adeno-MiNEN. Two of 5 (40%) acinar-MiNEN and one adeno-MiNEN patients had liver metastases. All but one adeno-MiNEN (88%) showed preoperative radiological features of pancreatic adenocarcinoma, 3 of 5 (60%) acinar-MiNEN exhibited mainly neuroendocrine features. No surgical mortality was observed. The 5-year overall survival rate in all MiNEN was 40%. Five-year survival rate was 58% in adeno-MiNEN and comparable to that of matched ductal adenocarcinomas (36%) and pNEN (48%). Five-year overall survival rate was 20% in acinar-MiNEN, compared to 39% in acinar carcinoma patients and 59% in matched pNEN patients.CONCLUSIONS: MiNEN are rare and difficult to distinguish from pure adenocarcinoma or neuroendocrine neoplasm preoperatively. Surgical resection would therefore be the treatment of choice in localized tumors.
KW - Adenocarcinoma/diagnosis
KW - Adult
KW - Aged
KW - Aged, 80 and over
KW - Carcinoma, Acinar Cell/diagnosis
KW - Carcinoma, Pancreatic Ductal/diagnosis
KW - Case-Control Studies
KW - Female
KW - Humans
KW - Kaplan-Meier Estimate
KW - Male
KW - Middle Aged
KW - Neoplasm Metastasis
KW - Neoplasms, Complex and Mixed/diagnosis
KW - Neuroendocrine Tumors/diagnosis
KW - Pancreatectomy
KW - Pancreatic Neoplasms/diagnosis
KW - Retrospective Studies
KW - Survival Analysis
KW - Treatment Outcome
U2 - 10.1016/j.pan.2020.11.020
DO - 10.1016/j.pan.2020.11.020
M3 - SCORING: Journal article
C2 - 33309225
VL - 21
SP - 224
EP - 235
JO - PANCREATOLOGY
JF - PANCREATOLOGY
SN - 1424-3903
IS - 1
ER -
