An Unusual Case of Large Cell Neuroendocrine Cancer of the Gallbladder With Mixed Adenocarcinoma Component in a Patient With Pancreatobiliary Maljunction

Sep 28, 2026

Gallbladder neuroendocrine tumors and neuroendocrine carcinomas are rare neuroendocrine neoplasms, often discovered incidentally or during evaluation of nonspecific symptoms. Standard treatment guidance and the role of adjuvant therapy are not well established due to limited case data. A 65-year-old South American woman presented with chronic intermittent post-prandial epigastric pain, bloating, nausea, and occasional diarrhea. Pancreatobiliary maljunction with dilation was identified, and primary gallbladder large cell neuroendocrine carcinoma with a minor adenocarcinoma component was diagnosed.

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CategoryHealthcare

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Pages13

Reading Time33 min

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Abstract

Introduction

Case Presentation

On examination and investigations

How to cite this article

Open Access Case Report DOI: 10.7759/cureus.37398 Review began 02/28/2023 Review ended 03/26/2023 Published 04/10/2023 © Copyright 2023 Fick et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0. , which permits unrestricted use , distribution , and reproduction in any medium , provided the original author and source are credited. An Unusual Case of Large Cell Neuroendocrine Cancer of the Gallbladder With Mixed Adenocarcinoma Component in a Patient With Pancreatobiliary Maljunction Anton Fick 1 , Kayla Tran 2 , Harsh Kandpal 3 , Matthew Burge 4 , Manju Chandrasegaram 1 1. General Surgery, The Prince Charles Hospital, Brisbane, AUS 2. Pathology, The Prince Charles Hospital, Brisbane, AUS 3. Radiology, The Prince Charles Hospital, Brisbane, AUS 4. Medical Oncology, The Prince Charles Hospital, Brisbane, AUS Corresponding author: Manju Chandrasegaram, [m.chandrasegaram@uq.edu.au](m.chandrasegaram@uq.edu.au) Abstract Gallbladder neuroendocrine tumors (GB-NETs) and gallbladder neuroendocrine carcinomas (GB-NECs) are rare forms of neuroendocrine neoplasms (NENs) . Most GB-NENs present as incidental findings or as gallbladder polyps in the course of investigation of nonspecific symptoms such as upper abdominal pain and nausea. Given the rarity of GB-NENs, only a few small case series are currently available describing this entity, and even fewer guiding consensus on standard treatment and the role of adjuvant therapy. We present the case of a 65-year-old South American female referred for a chronic history of intermittent post-prandial epigastric pain, bloating, nausea, and occasional diarrhea. Pancreaticobiliary maljunction with dilation was present and she was diagnosed with primary gallbladder large cell neuroendocrine carcinoma (GB-LCNEC) mixed with a minor component of gallbladder adenocarcinoma. Categories: General Surgery Keywords: large cell neuroendocrine tumour, pancreatobiliary maljunction, gallbladder neuroendocrine carcinoma, gallbladder neuroendocrine tumour, gallbladder neuroendocrine neoplasm Introduction Gallbladder cancers account for only 0.5% of all malignant cancers with an incidence of ~ 1.2 per 100,000 [1- 3] . Neuroendocrine neoplasms (NENs) are a group of rare heterogeneous tumors representing ~ 2% of all malignancies and are functionally divided into two groups based on their clinical behavior, histology, and proliferation rate. Neuroendocrine tumors (NETs) include low to intermediate-grade well-differentiated tumors, and neuroendocrine carcinomas (NECs) include high-grade poorly differentiated tumors [4,5] . Neuroendocrine neoplasms most commonly affect the gastrointestinal system accounting for ~ 66% of all NENs [2,5,6]. Rarer still are primary gallbladder neuroendocrine neoplasms (GB-NENs) comprising only 0.5% to 2% to 3% of all gallbladder cancers [1,7-9] and 0.2% to 0.5% of all NENs [2,5,8, 10]. The incidence of GBNENs is reported to be less than 0.74 per 100,000 while gallbladder neuroendocrine carcinomas (GB-NECs) account for only 0.2% of all gastrointestinal NECs (GI-NECs), 0.5% of all NECs, and 2 . 1% of all gallbladder carcinomas [5,6] . Gallbladder neuroendocrine neoplasms are thought to arise from multipotent stem cells or neuroendocrine cells following gastric or intestinal metaplasia as a consequence of chronic inflammation secondary to gallstones [1,2,5,8, 10-13]. Gallbladder neuroendocrine neoplasms were reported as mixed in four of 10 cases reported in a case series [6] . A retrospective review of the American National Cancer Database by Ayabe et [al. in 2019](al. in 2019), identified 754 patients with GB-NET. Of these, 480 (64%) underwent resection with most undergoing a simple cholecystectomy (n=431, 90%) . A minority received multimodal therapy (n=145, 21%) . Patients who were older and had large cell histology or positive margins had poorer survival following surgery [9] . Case Prese

FAQs

What are gallbladder neuroendocrine tumors and neuroendocrine carcinomas, and why are they challenging to manage?

They are rare neuroendocrine neoplasms. Limited case series mean consensus on standard treatment and the role of adjuvant therapy remains unclear.

What symptoms did the 65-year-old patient have at presentation?

She reported intermittent post-prandial epigastric pain, bloating, nausea, and occasional diarrhea over a chronic course.

What diagnostic findings led to the final cancer diagnosis?

Imaging showed focal gallbladder wall thickening suspicious for neoplasia, and the patient had elevated CA19-9. The workup supported primary gallbladder large cell neuroendocrine carcinoma with a minor adenocarcinoma component in the setting of pancreatobiliary maljunction.

Sep 28, 2026

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