Department of Special Treatment and Liver Transplantation, Eastern Hepatobiliary Surgery Hospital, Second Military Medical University, Shanghai 200438, China.
Hepatobiliary Pancreat Dis Int. 2010 Feb;9(1):44-8.
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.
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.
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 post-operative 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).
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.
原发性肝神经内分泌癌（PHNEC）极为罕见，英文和中文文献中报道的病例不足 300 例，因此难以做出正确诊断和确定治疗方法。
1996 年 1 月至 2008 年 5 月期间，我院收治了 11 例 PHNEC 患者。对患者进行实验室检查、消化内镜、B 超、CT、MRI 或 PET-CT 检查以进行术前诊断。所有患者均接受了肝切除术。部分患者在发现复发时接受了经导管动脉化疗栓塞术（TACE）、经皮乙醇注射治疗（PEIT）或奥曲肽注射治疗。记录患者的临床资料并对所有患者进行随访。
患者经病理检查确诊为 PHNEC。中位随访时间为 33 个月（12-107 个月）。所有患者均存活，最长术后生存时间为 107 个月，最长无病生存时间为 98 个月，1 年生存率为 100%，1 年复发率为 45.5%（5/11）。
由于 PHNEC 容易与肝细胞癌混淆，因此需要仔细筛查症状以避免误诊。肝切除术是 PHNEC 的首选治疗方法，可提供包括长期生存在内的最有利结局。也可以考虑其他治疗方法，如 TACE 和 PEIT，特别是在肿瘤复发时。
