Lan Jianwei, Guo Deliang, Qin Xian, Chen Baiyang, Liu Quanyan
Department of Hepatobiliary and Pancreatic Surgery, Research Center of Digestive Diseases, Zhongnan Hospital of Wuhan University, Wuhan, China.
Department of General Surgery, Xiangyang Central Hospital, Affiliated of Hubei University of Arts and Science, Xiangyang, China.
Front Surg. 2021 Jul 14;8:678853. doi: 10.3389/fsurg.2021.678853. eCollection 2021.
Neuroendocrine tumors are heterogeneous malignancies that originate from the neuroendocrine system. Previous studies show that this cancer type mainly localizes in the gastrointestinal tract and often metastasizes to the liver. Primary liver neuroendocrine tumors are very rare and primary hepatic neuroendocrine tumors (PHNET) with concurrent hepatocellular carcinoma (HCC) are extremely rare. To the best of our knowledge, only few PHNET cases have been identified, making their diagnosis difficult. Here, we report the biggest ever reported and "deceiving" lesion of a mixed neuroendocrine-non-neuroendocrine neoplasm in the liver, aiming to raise awareness and improve treatment of the disease. Here, we report a preoperative misdiagnosed case that presented with hepatocellular carcinoma clinical features and no extrahepatic tumors. Postoperative pathology confirmed that it was a mixed neuroendocrine-non-neuroendocrine neoplasm. The patient was then referred for etoposide and cisplatin-based chemotherapy. No disease recurrence was observed at the 6-month follow-up. We report a very rare and easily misdiagnosed case and we speculate that there were "undifferentiated cells" undergoing neuroendocrine and hepatocellular carcinoma differentiation, during which some hepatocellular carcinoma cells express neuroendocrine features. We recommend proper surgery and postoperative platinum-based chemotherapy in the management of this disease.
神经内分泌肿瘤是起源于神经内分泌系统的异质性恶性肿瘤。先前的研究表明，这种癌症类型主要位于胃肠道，且常转移至肝脏。原发性肝脏神经内分泌肿瘤非常罕见，而同时合并肝细胞癌（HCC）的原发性肝神经内分泌肿瘤（PHNET）极为罕见。据我们所知，仅发现少数PHNET病例，这使得其诊断困难。在此，我们报告了肝脏中最大的一例已报道的“具有欺骗性”的混合性神经内分泌-非神经内分泌肿瘤病变，旨在提高对该疾病的认识并改善其治疗。在此，我们报告一例术前误诊病例，该病例表现出肝细胞癌的临床特征且无肝外肿瘤。术后病理证实为混合性神经内分泌-非神经内分泌肿瘤。随后该患者接受了依托泊苷和顺铂为主的化疗。在6个月的随访中未观察到疾病复发。我们报告了一例非常罕见且容易误诊的病例，我们推测存在正在经历神经内分泌和肝细胞癌分化的“未分化细胞”，在此过程中一些肝细胞癌细胞表达神经内分泌特征。我们建议在该疾病的管理中进行适当的手术及术后铂类化疗。
