Mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater: a case report

Abstract Background Mixed neuroendocrine–non-neuroendocrine neoplasms of the ampulla of Vater are rare and heterogenous, making it difficult to achieve a definitive preoperative diagnosis. Herein, we describe a patient in whom a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neopla...

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Main Authors: Kenjiro Date, Takaaki Tatsuguchi, Yuzo Shimokawa, Yusuke Niina, Daichi Kitahara, Hirotaka Kuga, Sadafumi Tamiya, Kazuyoshi Nishihara, Toru Nakano
Format: Article
Language:English
Published: Japan Surgical Society 2023-06-01
Series:Surgical Case Reports
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Online Access:https://doi.org/10.1186/s40792-023-01689-6
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author Kenjiro Date
Takaaki Tatsuguchi
Yuzo Shimokawa
Yusuke Niina
Daichi Kitahara
Hirotaka Kuga
Sadafumi Tamiya
Kazuyoshi Nishihara
Toru Nakano
author_facet Kenjiro Date
Takaaki Tatsuguchi
Yuzo Shimokawa
Yusuke Niina
Daichi Kitahara
Hirotaka Kuga
Sadafumi Tamiya
Kazuyoshi Nishihara
Toru Nakano
author_sort Kenjiro Date
collection DOAJ
description Abstract Background Mixed neuroendocrine–non-neuroendocrine neoplasms of the ampulla of Vater are rare and heterogenous, making it difficult to achieve a definitive preoperative diagnosis. Herein, we describe a patient in whom a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater was made preoperatively. Case presentation Computed tomography revealed an enhancing periampullary tumor in a 69-year-old man with obstructive jaundice. Subsequent duodenoscopy revealed an ulcerated lesion in the swollen ampulla of Vater, from which six biopsies were collected. Pathological examination revealed adenocarcinoma in five of them. The remaining one was a neuroendocrine neoplasm according to immunohistochemical analysis. With a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater, the patient underwent subtotal stomach-preserving pancreaticoduodenectomy with modified Child’s reconstruction and was discharged without complications. Pathological examination revealed both adenocarcinoma and neuroendocrine carcinomas, each accounting for ≥ 30% of the tumor, resulting in a definitive diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater. Lymph node metastases with neuroendocrine components were also observed. Adjuvant chemotherapy was not administered because of the patient’s renal dysfunction. Liver and lymph node metastases were detected 2 months after surgery, the neuroendocrine component being considered responsible for that relapse. The patient underwent platinum-based chemotherapy at 50% dosage, which initially resulted in significant tumor shrinkage; however, he died 6 months after surgery. Conclusions While these tumors’ heterogeneity make definitive preoperative diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater difficult, the possibility of this disease can be considered by careful examination. Further study is needed to establish the optimal diagnostic criteria and treatment strategy.
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spelling doaj-art-df97ac981d5e4e98b761bbfb4e1a198a2025-08-20T02:51:04ZengJapan Surgical SocietySurgical Case Reports2198-77932023-06-01911510.1186/s40792-023-01689-6Mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater: a case reportKenjiro Date0Takaaki Tatsuguchi1Yuzo Shimokawa2Yusuke Niina3Daichi Kitahara4Hirotaka Kuga5Sadafumi Tamiya6Kazuyoshi Nishihara7Toru Nakano8Department of Surgery, Kitakyushu Municipal Medical CenterDepartment of Surgery, Kitakyushu Municipal Medical CenterDepartment of Gastroenterology, Kitakyushu Municipal Medical CenterDepartment of Gastroenterology, Kitakyushu Municipal Medical CenterDepartment of Pathology, Kitakyushu Municipal Medical CenterDepartment of Surgery, Kitakyushu Municipal Medical CenterDepartment of Pathology, Kitakyushu Municipal Medical CenterDepartment of Surgery, Kitakyushu Municipal Medical CenterDepartment of Surgery, Kitakyushu Municipal Medical CenterAbstract Background Mixed neuroendocrine–non-neuroendocrine neoplasms of the ampulla of Vater are rare and heterogenous, making it difficult to achieve a definitive preoperative diagnosis. Herein, we describe a patient in whom a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater was made preoperatively. Case presentation Computed tomography revealed an enhancing periampullary tumor in a 69-year-old man with obstructive jaundice. Subsequent duodenoscopy revealed an ulcerated lesion in the swollen ampulla of Vater, from which six biopsies were collected. Pathological examination revealed adenocarcinoma in five of them. The remaining one was a neuroendocrine neoplasm according to immunohistochemical analysis. With a provisional diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater, the patient underwent subtotal stomach-preserving pancreaticoduodenectomy with modified Child’s reconstruction and was discharged without complications. Pathological examination revealed both adenocarcinoma and neuroendocrine carcinomas, each accounting for ≥ 30% of the tumor, resulting in a definitive diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater. Lymph node metastases with neuroendocrine components were also observed. Adjuvant chemotherapy was not administered because of the patient’s renal dysfunction. Liver and lymph node metastases were detected 2 months after surgery, the neuroendocrine component being considered responsible for that relapse. The patient underwent platinum-based chemotherapy at 50% dosage, which initially resulted in significant tumor shrinkage; however, he died 6 months after surgery. Conclusions While these tumors’ heterogeneity make definitive preoperative diagnosis of mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater difficult, the possibility of this disease can be considered by careful examination. Further study is needed to establish the optimal diagnostic criteria and treatment strategy.https://doi.org/10.1186/s40792-023-01689-6AdenocarcinomaAmpulla of VaterMixed neuroendocrine–non-neuroendocrine neoplasmNeuroendocrine carcinoma
spellingShingle Kenjiro Date
Takaaki Tatsuguchi
Yuzo Shimokawa
Yusuke Niina
Daichi Kitahara
Hirotaka Kuga
Sadafumi Tamiya
Kazuyoshi Nishihara
Toru Nakano
Mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater: a case report
Surgical Case Reports
Adenocarcinoma
Ampulla of Vater
Mixed neuroendocrine–non-neuroendocrine neoplasm
Neuroendocrine carcinoma
title Mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater: a case report
title_full Mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater: a case report
title_fullStr Mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater: a case report
title_full_unstemmed Mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater: a case report
title_short Mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater: a case report
title_sort mixed neuroendocrine non neuroendocrine neoplasm of the ampulla of vater a case report
topic Adenocarcinoma
Ampulla of Vater
Mixed neuroendocrine–non-neuroendocrine neoplasm
Neuroendocrine carcinoma
url https://doi.org/10.1186/s40792-023-01689-6
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