Neuroendocrine Carcinoma of Gall Bladder - A Case Series and Review of Literature

Introduction: Neuroendocrine carcinoma (NEC) of the gallbladder is an exceptionally rare and aggressive malignancy, representing only 0.2% of all gastrointestinal and hepatobiliary NECs. Due to its rarity, evidence - based guidelines for diagnosis and treatment remain undefined. Methodology: We conducted a retrospective analysis of NEC of the gallbladder cases diagnosed at our institute from 2005 to 2023. Clinical records, imaging, histopathological reports, and treatment details were reviewed to characterize presentation, management, and outcomes. Results: A total of 10 cases were identified. The median age at diagnosis was 48.5 years (range 18?85), with a female predominance (M: F = 1: 4). Most patients presented with abdominal pain and weight loss. Imaging with contrast - enhanced CT revealed locally advanced disease in 6 patients and metastatic disease in 4. One patient was incidentally diagnosed post - cholecystectomy. Diagnosis was confirmed via histopathology and immunohistochemistry, with synaptophysin and chromogranin positivity in 90% and 80% of cases respectively. The Ki - 67 index was >80% in 9 cases. Six patients received etoposide - platinum chemotherapy, with a median progression - free survival of 5.5 months. Progression included hepatic dysfunction, brain metastasis, and radiological advancement. Conclusion: Gallbladder NECs are typically diagnosed at an advanced stage due to non - specific symptoms. The absence of standardized treatment protocols underscores the importance of individualized, multidisciplinary approaches. Further research and clinical trials are warranted to establish optimal therapeutic strategies.