Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma

Background: Large cell neuroendocrine carcinoma (LCNEC) of the liver, gallbladder, pancreas, and ampulla is rare and usually arises in case reports, and thus to date, no studies have well described the treatment options and outcomes of those patients.Methods: The data of 108 patients diagnosed as hepatopancreatobiliary and ampulla LCNEC between 2004 and 2015 were retrieved from the surveillance, epidemiology, and final results.Results: In the entire cohort, the median overall survival (OS) was 10 months. For nonmetastatic patients, the median OS was 32 months for surgery of the primary tumor alone (n = 17), 19 months for surgery of the primary tumor and adjuvant therapy (n = 19), and 1 month for nonsurgical treatment (n = 8). For metastatic patients, the median OS was 14 months for patients who received surgery of the primary tumor with and without adjuvant therapy (n = 16), 9 months for patients undergoing adjuvant treatment alone (n = 30), and 1 month for patients who had no treatment (n = 16). Multivariate analysis revealed that surgery of the primary tumor was an independent factor for improved survival.Conclusions: This disease offers a very poor prognosis despite aggressive treatment. Radical resection is the first choice for resectable tumors, whereas surgical resection of the primary tumor plus adjuvant therapy might represent a valid option for metastatic disease. However, further studies are needed to confirm this.