Shah Darshil, Mandot Ameet, Cerejo Clancy, Amarapurkar Deepak, Pal Anil
Gastroenterology, Bombay Hospital Gastroenterology, 402, Pawan Building, Indraprasth Complex, Satya Nagar, Borivali(West) Mumbai, Maharashtra, 400092, India.
DNB Gastroenterology, Bombay Hospital, India.
J Clin Exp Hepatol. 2019 Nov-Dec;9(6):710-715. doi: 10.1016/j.jceh.2019.08.002. Epub 2019 Aug 16.
Primary hepatic neuroendocrine tumor is an extremely rare entity. Only case reports are available in the literature. The aim of the study was to analyze the symptoms, diagnosis, management, and the outcome of patients with primary liver neuroendocrine tumors.
In the study, a total of eight patients were diagnosed with primary liver neuroendocrine tumors between 2001 and 2017 in our center. Data were analyzed from the records available including the presentation, diagnosis, treatment received, and follow-up.
Of eight patients, five were males and three were females. The age of presentation was between 35 and 70 years. Two patients had pain in the right side of the abdomen, while it was accidentally detected in two patients in routine checkup. One patient presented with carcinoid syndrome, while two had ascites and one patient presented only with loose motions. Of eight patients, two patients with poorly differentiated neuroendocrine tumor died within 1 month of follow-up. Four patients are still being followed up, while 10-12 years of follow-up data are available for the remaining two patients. Four patients underwent surgery, and three patients received Sandostatin LAR for tumor recurrence after procedure. Transarterial chemoembolization (TACE) of the tumor was performed in two patients for whom resection was not possible.
Our data suggest that the prognosis of the tumor seems favorable. Surgical resection is the curative treatment. TACE is a favorable option in unresectable tumors.
原发性肝神经内分泌肿瘤是一种极其罕见的疾病。文献中仅有病例报告。本研究的目的是分析原发性肝神经内分泌肿瘤患者的症状、诊断、治疗及预后。
本研究纳入了2001年至2017年间在我们中心诊断为原发性肝神经内分泌肿瘤的8例患者。分析现有记录中的数据，包括临床表现、诊断、接受的治疗及随访情况。
8例患者中，5例为男性，3例为女性。发病年龄在35至70岁之间。2例患者有右侧腹痛，2例在常规检查中偶然发现。1例患者出现类癌综合征，2例有腹水，1例仅表现为腹泻。8例患者中，2例低分化神经内分泌肿瘤患者在随访1个月内死亡。4例患者仍在随访中，其余2例患者有10至12年的随访数据。4例患者接受了手术，3例患者术后因肿瘤复发接受了长效奥曲肽治疗。2例无法切除肿瘤的患者接受了经动脉化疗栓塞（TACE）。
我们的数据表明该肿瘤的预后似乎良好。手术切除是治愈性治疗方法。TACE是不可切除肿瘤的一种理想选择。
