Chen Nelson, Slater Kellee
Department of Hepatobiliary and Pancreatic Surgery, Princess Alexandra Hospital, 199 Ipswich Road, Woolloongabba, Queensland, 4102, Australia; University of Queensland, Australia.
Department of Hepatobiliary and Pancreatic Surgery, Greenslopes Private Hospital, Newdegate Street, Greenslopes, Queensland, 4102, Australia.
Int J Surg Case Rep. 2019;55:145-148. doi: 10.1016/j.ijscr.2019.01.025. Epub 2019 Jan 30.
Primary hepatic neuroendocrine tumours (PHNET) were first described by Edmondson et al. in 1958 and are rare, accounting for only 0.3% of all neuroendocrine tumours. Only several hundred cases have been reported.
We present two cases. The first is a 65-year-old asymptomatic male referred with a liver lesion on ultrasound performed to investigate a mildly elevated Alanine Aminotransferase (ALT). Hepatitis serology and tumour markers were normal. He had an unremarkable colonoscopy and gastroscopy. CT and MRI revealed a single liver lesion adjacent to the gallbladder suspicious for malignancy. He underwent a segment IVb/V liver resection. Histology was consistent with a 65 mm grade 2 PHNET. Subsequent Dotatate PET/CT scans have been normal at 5 years. The second is an asymptomatic 73-year-old male referred with fluctuating hepatic enzymes and a history of alcohol overuse. Imaging revealed a suspicious lesion in segment III of the liver. He underwent a left lateral liver resection. Histology revealed an 18 mm grade 1 PHNET. A subsequent Dotatate PET/CT was normal with no new disease at six months.
PHNET, albeit rare are in the differential diagnosis for primary hepatic malignancies. Tumour markers are usually normal and radiological imaging can mimic other hypervascular hepatic tumours. Surgery is the only curative treatment for localised disease to date.
PHNET needs to be considered in asymptomatic patients with hypervascular hepatic lesions. More research is required before other adjunct treatment options can be suggested.
原发性肝神经内分泌肿瘤(PHNET)最早由埃德蒙森等人于1958年描述,较为罕见,仅占所有神经内分泌肿瘤的0.3%。仅报道过几百例病例。
我们呈现两例病例。第一例是一名65岁无症状男性,因丙氨酸转氨酶(ALT)轻度升高行超声检查发现肝脏病变而就诊。肝炎血清学和肿瘤标志物均正常。他的结肠镜检查和胃镜检查均无异常。CT和MRI显示胆囊旁有一个单一肝脏病变,怀疑为恶性肿瘤。他接受了IVb/V段肝切除术。组织学检查结果与一个65毫米的2级PHNET一致。随后的镓[68Ga] DOTATATE PET/CT扫描在5年时均正常。第二例是一名73岁无症状男性,因肝酶波动和有酗酒史而就诊。影像学检查显示肝脏III段有一个可疑病变。他接受了左外侧肝切除术。组织学检查显示为一个18毫米的1级PHNET。随后的镓[68Ga] DOTATATE PET/CT在6个月时正常,无新发病灶。
PHNET虽然罕见,但在原发性肝恶性肿瘤的鉴别诊断中需要考虑。肿瘤标志物通常正常,影像学检查可能与其他高血供肝脏肿瘤相似。手术是目前针对局限性疾病的唯一治愈性治疗方法。
对于有高血供肝脏病变的无症状患者,需要考虑PHNET。在能够提出其他辅助治疗方案之前,还需要更多的研究。