Department of Internal Medicine, Yonsei Institute of Gastroenterology, Yonsei, Korea.
J Gastroenterol Hepatol. 2012 Aug;27(8):1306-11. doi: 10.1111/j.1440-1746.2012.07117.x.
Primary hepatic neuroendocrine carcinomas (PHNECs) are extremely rare, with only about 150 cases having been reported in the English-language literature. Because of the rarity of PHNECs, its clinical features and treatment outcomes are not well understood. Here, we report our experiences with PHNECs.
We identified patients diagnosed with PHNEC and analyzed their demographics, baseline laboratory data, tumor characteristics, treatment modalities and outcomes.
A total of 218 consecutive patients were identified with pathologically confirmed neuroendocrine carcinoma. Of these, 12 patients were diagnosed with PHNECs; the median age was 66.5 years (range, 37 to 80 years), and seven patients (58.3%) were male. Two patients who each had a single hepatic mass underwent curative surgical resection. One patient who was of inoperable status at the initial diagnosis because of multiple intrahepatic metastases showed a partial response after the ninth round of systemic chemotherapy and then underwent surgical resection. The median overall survival in the 12 patients was 16.5 months (range, 0.7 to 41.7 months). Three patients who underwent surgical treatment are alive without recurrence for 15.2 months, 18.0 months, and 36.9 months, respectively.
Primary hepatic neuroendocrine carcinoma should be considered as a possible differential diagnosis in the management of hepatic tumors. The liver can be the primary origin of neuroendocrine tumors, and if the tumors are diagnosed as primary hepatic neuroendocrine tumors, surgical resection must be considered for curative treatment.
原发性肝神经内分泌癌（PHNEC）极为罕见，英文文献中仅报道了约 150 例。由于 PHNEC 罕见，其临床特征和治疗结果尚不清楚。在此，我们报告我们的 PHNEC 经验。
我们确定了诊断为 PHNEC 的患者，并分析了他们的人口统计学、基线实验室数据、肿瘤特征、治疗方式和结果。
共确定了 218 例经病理证实为神经内分泌癌的连续患者；其中 12 例患者被诊断为 PHNEC；中位年龄为 66.5 岁（范围，37 至 80 岁），7 例（58.3%）为男性。两名患者各有一个单一的肝肿块，接受了根治性手术切除。一名患者在最初诊断时因多发肝内转移而无法手术，在进行了 9 轮全身化疗后显示部分缓解，然后接受了手术切除。12 例患者的中位总生存期为 16.5 个月（范围，0.7 至 41.7 个月）。接受手术治疗的 3 例患者分别在无复发的情况下存活了 15.2 个月、18.0 个月和 36.9 个月。
原发性肝神经内分泌癌在肝脏肿瘤的治疗中应被视为可能的鉴别诊断。肝脏可能是神经内分泌肿瘤的原发部位，如果肿瘤被诊断为原发性肝神经内分泌癌，必须考虑手术切除作为治愈性治疗。
