Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Dec 6, 2019; 7(23): 4119-4129
Published online Dec 6, 2019. doi: 10.12998/wjcc.v7.i23.4119
Mixed serous-neuroendocrine neoplasm of the pancreas: A case report and review of the literature
Yue-Mei Xu, Zhi-Wen Li, Hong-Yan Wu, Xiang-Shan Fan, Qi Sun
Yue-Mei Xu, Zhi-Wen Li, Hong-Yan Wu, Xiang-Shan Fan, Qi Sun, Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing 210008, Jiangsu Province, China
Author contributions: Xu YM and Sun Q were the patient’s diagnostic pathologists, followed the patient, reviewed the literature, and contributed to manuscript drafting; Li ZW collected the data and contributed to manuscript drafting; Wu HF was responsible for immunohistochemical detection; Sun Q and Fan XS were responsible for the revision of the manuscript for important intellectual content; all authors issued final approval for the version to be submitted.
Supported by National Natural Science Foundation of China, No. 81702596.
Informed consent statement: Written informed consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Qi Sun, MD, Doctor, Department of Pathology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, Jiangsu Province, China. blkqisun@qq.com
Telephone: +86-25-83106666-10306 Fax: +86-25-83106666-10306
Received: August 22, 2019
Peer-review started: August 22, 2019
First decision: September 23, 2019
Revised: October 14, 2019
Accepted: November 15, 2019
Article in press: November 15, 2019
Published online: December 6, 2019
Processing time: 106 Days and 5.2 Hours
Abstract
BACKGROUND

Pancreatic mixed serous-neuroendocrine neoplasms (MSNNs) are mixed tumors containing two components with different pathologies, namely, pancreatic serous cystic neoplasm (PSCN) and pancreatic neuroendocrine tumor (PanNET). For MSNNs, diffuse PSCN involving the whole pancreas is extremely rare, with only eight previous case reports.

CASE SUMMARY

A 45-year-old Chinese woman, with a free previous medical history and no obvious symptoms, was found to have a pancreatic neoplasm and admitted to our hospital for further diagnosis in March 2018. Abdominal palpation revealed a painless, mobile mass in the epigastrium, and no abnormalities were observed in an examination of the nervous system and ocular system. A computed tomography scan showed multiple cystic lesions involving the whole pancreas ranging in diameter from 0.4 to 2 cm and also revealed an enhanced mass, 2.2 cm in diameter, in the head of the pancreas. Moreover, multiple cysts were found in the kidneys bilaterally, and the right lobe of the liver contained a small cyst. A Whipple operation with total pancreatectomy and splenectomy was performed. A diagnosis of pancreatic MSNN was established, consisting of diffuse serous microcystic cystadenoma with a concomitant grade 2 PanNET. Of note, the patient had no personal or family history of Von Hippel-Lindau syndrome or other disease.

CONCLUSION

We report the first case of MSNN with a diffuse PSCN component involving the entire pancreas in a Chinese woman. It is important to be aware of its relationship with VHL syndrome, and close clinical follow-up is recommended.

Keywords: Mixed serous-neuroendocrine neoplasm; Pancreatic serous cystic neoplasm; Pancreatic neuroendocrine tumor; Von Hippel-Lindau syndrome; Case report

Core tip: Mixed serous-neuroendocrine neoplasm (MSNN). MSNNs are a mixed tumor containing two components with different pathologies, namely pancreatic serous cystic neoplasm (PSCN) and pancreatic neuroendocrine tumor (PanNET). For MSNNs, diffuse PSCN involving the whole pancreas is extremely rare with only eight previous case reports. Here, we report a novel case of diffuse PSCN admixture with an isolated PanNET in a Chinese woman, and also review the previous literature. Due to its close relationship with Von Hippel-Lindau syndrome, and the malignant potential of neuroendocrine component, patients with MSNN should undergo thorough systemic examinations, including molecular genetic analysis, and close clinical follow-up is also recommended.