ABSTRACT
Neuroendocrine tumors of the gallbladder(GB-NET) are rare, and it lacks early clinical manifestations and has no specific tumor markers, it is difficult to distinguish GB-NET from gallbladder adenocarcinoma. The diagnosis of GB-NET is based on histopathology of the tumor and the assessment of proliferation fraction, which makes it difficult to achieve early diagnosis. GB-NET has a high degree of malignancy, 32.39% of patients have liver metastases at diagnosis, and 51.60% of patients have lymph node metastases, the median survival time is 9 to 10 months.There are currently no specific guidelines or consensus for the treatment of GB-NET. The treatment strategies are choosen mainly by the principles of gallbladder adenocarcinoma. We reviews the clinical and basic researches of GB-NET and case reports from China and across the world, as well as the data from SEER database, and we discuss the research progress on the classification, clinicopathological features, diagnosis, treatment advances and the prognosis.