AbstractBackgroundNeuroendocrine neoplasm (NEN) of the gallbladder is rare. It is usually asymptomatic and occurs in older adults. Its clinicopathological characteristics remain controversial, and the diagnosis and treatment strategies are usually based on models of adenocarcinoma. The present study aimed to investigate the clinical characteristics and prognosis of gallbladder NEN.MethodsThe data of patients with gallbladder NEN admitted to Peking Union Medical College Hospital was reviewed, and a database was established for retrospective analysis. Clinicopathological features were analyzed descriptively and the prognosis was studied according to different factors. The Kaplan–Meier curve was used to describe the cumulative survival rate.ResultsIn total, 22 patients with gallbladder NEN were included in this study. There were 10 male (45.5%) and 12 female (54.5%) patients with a median age of onset of NEN at 57.5 (49.0, 62.3) years. Abdominal discomfort was the most common symptom. Twenty patients (90.9%) underwent surgery, and two patients (9.1%) with unresectable lesions underwent a biopsy. Twenty‐one patients were followed up. The 1‐, 2‐, and 3‐year cumulative overall survival rates of all patients and patients with resectable lesions were 65.9%, 54.9%, and 48.1%, and 72.9%, 60.7%, and 53.1%, respectively. Patients with resectable lesions had a better cumulative overall survival rate than those who with unresectable lesions (p< 0.001).ConclusionGallbladder NEN is more common in the elderly and has a slight female predominance. The most common symptom is abdominal discomfort. Surgery is the first choice of treatment for this rare disease. The prognosis of gallbladder NEN is generally poor. Patients with resectable lesions have a better prognosis.