Abstract

BackgroundGallbladder neuroendocrine tumors (GB-NETs) are extremely rare, representing only 0.5% of all NETs because no neuroectodermal cells are present in the gallbladder. In 2019, the World Health Organization updated the classification of NETs based on their molecular differences. The mutation status of DAXX and ATRX has been added to the criteria for well-differentiated NETs.Case presentationA 50-year-old man presented to our hospital for further examination of a gallbladder polyp. He had no right quadrant pain, fever, jaundice, weight loss, or carcinoid syndrome-related symptoms. The patient hoped to avoid cholecystectomy. During the 3-year observation period, the polyp gradually increased in size from 8.3 to 9.9 mm. He decided to undergo surgery, and whole cholecystectomy was successfully performed. Immunohistochemical staining revealed positivity for chromogranin A, synaptophysin, and CD56. The Ki-67 index was < 3%. Taken together, these results led to a diagnosis of a grade 1 GB-NET. We also performed immunohistochemical staining of DAXX and ATRX, which revealed that DAXX protein expression was negative. The patient’s postoperative course was uneventful, and he developed no recurrence for 8 years after surgery.ConclusionWe experienced a very rare case of GB-NET. Obtaining a correct preoperative diagnosis is quite difficult at the first evaluation. A GB-NET should be considered as a differential diagnosis of gallbladder tumors.

Highlights

  • Gallbladder neuroendocrine tumors (GB-NETs) are extremely rare, representing only 0.5% of all NETs because no neuroectodermal cells are present in the gallbladder

  • In the last 20 years the definition of carcinoid has changed to NET

  • In 2010, the World Health Organization (WHO) updated the classification of NETs into three new categories according to the proliferative ability of the tumor: grade 1, 2, and 3 NETs [8]

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Summary

Introduction

Gallbladder neuroendocrine tumors (GB-NETs) are extremely rare, representing only 0.5% of all NETs because no neuroectodermal cells are present in the gallbladder. A GB-NET should be considered as a differential diagnosis of gallbladder tumors. Multipotent stem cells or neuroendocrine cells involved in intestinal or gastric metaplasia of the gallbladder epithelium have recently been considered the origin of GB-NETs [3].

Results
Conclusion

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