BACKGROUND:Primary hepatic neuroendocrine carcinoma (PHNEC) is extremely rare,and fewer than 300 cases have been reported in the English/Chinese-language literature,therefore it is difficult to make a proper diagnosis and determine a therapeutic approach.METHODS:Eleven PHNEC patients were admitted to our hospital between January 1996 and May 2008.Laboratory examination,digestive endoscopy,B-ultrasonography,CT,MRI,or PET-CT were performed on the patients for preoperative diagnosis.All patients received liver resection.Some patients received transcatheter arterial chemoembolization (TACE),percutaneous ethanol injection treatment (PEIT),or octreotide injection when a recurrence was found.The patients’ clinical data were recorded and all patients were followed up.RESULTS:The patients were confirmed pathologically as having PHNEC.Their median follow-up time was 33 months (12-107 months).All patients survived,and the longest postoperative survival time was 107 months,the longest diseasefree survival time was 98 months,the 1-year survival rate was 100%,and the 1-year recurrence rate was 45.5% (5/11).CONCLUSIONS:Since PHNEC is easy to confuse with hepatocellular carcinoma,careful screening of symptoms is needed to avoid misdiagnosis.Resection is the first choice of treatment for PHNEC and provides the most favorable outcomes including long-term survival.Other treatment such as TACE and PEIT can be considered as well,especially when a tumor recurs. BACKGROUND: Primary hepatic neuroendocrine carcinoma (PHNEC) is extremely rare, and fewer than 300 cases have been reported in the English / Chinese-language literature, therefore it is difficult to make proper diagnosis and determine a therapeutic approach. METHODS: Eleven PHNEC patients were admitted to our hospital between January 1996 and May 2008. Laboratory examination, digestive endoscopy, B-ultrasonography, CT, MRI, or PET-CT were performed on the patient for preoperative diagnosis. All patients received liver resection. Patients received transcatheter arterial The chemoembolization (TACE), orcutaneous injection of PEIT, or octreotide injection when a recurrence was found. the patients’ clinical data were recorded and all patients were followed up .RESULTS: The patients were confirmed pathologically as having PHNEC.Their median follow -up time was 33 months (12-107 months). All patients survived, and the longest postoperative survival time was 107 months, the longest disease free survival time was 98 months, the 1-year survival rate was 100%, and the 1-year recurrence rate was 45.5% (5/11) .CONCLUSIONS: Since PHNEC is easy to confuse with hepatocellular carcinoma, careful screening of symptoms is needed to avoid misdiagnosis. Recovery is the first choice of treatment for PHNEC and provides the most favorable outcomes including long-term survival. Other treatment such as TACE and PEIT can be considered as well, especially when a tumor recurs.