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Diagnostic Pathology
Published in: Diagnostic Pathology 1/2021

Open Access 01-12-2021 | Neuroendocrine Tumor | Case Report

Case report: composite pancreatic intraductal papillary mucinous neoplasm and neuroendocrine tumor: a new mixed neuroendocrine-non-neuroendocrine neoplasm?

Authors: Jingci Chen, Pengyan Wang, Ke Lv, Weixun Zhou

Published in: Diagnostic Pathology | Issue 1/2021

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Abstract

Background

Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) of the pancreas are extremely rare. Their pathogenesis and molecular landscape are largely unknown. Here, we report a case of mixed pancreatic intraductal papillary mucinous neoplasm (IPMN) and well-differentiated neuroendocrine tumor (NET) and identify its genetic alterations by next-generation sequencing (NGS).

Case presentation

A fifty-year-old male was admitted into the hospital for evaluation of a pancreatic lesion detected during a routine examination. Abdominal ultrasound indicated a hypoechoic mass of 2.6 cm at the head of the pancreas. Malignancy was suspected and partial pancreatectomy was performed. Thorough histopathological examination revealed a mixed IPMN-NET. In some areas, the two components were relatively separated, whereas in other areas IPMN and NET grew in a composite pattern: The papillae were lined with epithelial cells of IPMN, and there were clusters of NET nests in the stroma of papillary axis. NGS revealed shared somatic mutations (KRAS, PCK1, MLL3) in both components. The patient has been uneventful 21 months after the surgery.

Conclusions

Our case provides evidence of a common origin for mixed IPMN-NET with composite growth features. Our result and literature review indicate that KRAS mutation might be a driver event underlying the occurrence of MiNEN. We also recommend the inclusion of mixed non-invasive exocrine neoplasms and neuroendocrine neoplasms into MiNEN.
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Metadata
Title
Case report: composite pancreatic intraductal papillary mucinous neoplasm and neuroendocrine tumor: a new mixed neuroendocrine-non-neuroendocrine neoplasm?
Authors
Jingci Chen
Pengyan Wang
Ke Lv
Weixun Zhou
Publication date
01-12-2021
Publisher
BioMed Central
Published in
Diagnostic Pathology / Issue 1/2021
Electronic ISSN: 1746-1596
DOI
https://doi.org/10.1186/s13000-021-01165-5

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