Background: Mixed neuroendocrine–non-neuroendocrine tumors (MINEN) of the liver are exceptionally rare, with limited data available regarding their clinical behavior, pathogenesis, and optimal management. The coexistence of hepatocellular carcinoma (HCC) and neuroendocrine carcinoma (NEC) within the liver presents diagnostic and therapeutic challenges. Methods: A systematic literature search was conducted on PubMed, identifying cases of primary mixed HCC and NEC in the liver. The search adhered to PRISMA guidelines, and relevant studies were critically analyzed. A total of 45 documented cases were reviewed, focusing on patient demographics, clinical characteristics, treatment strategies, and outcomes. Results: Most patients (90%) were male, with a median age of 66.5 years. Hepatitis B or C infection was present in 74% of cases, and liver cirrhosis was reported in 38%. The combined type was the most frequently observed histological pattern (65%). Treatment modalities varied, including transarterial chemoembolization (TACE), radiofrequency ablation (RFA), surgery, and systemic therapies. The median overall survival was 10 months, highlighting the aggressive nature of these tumors. Conclusions: Given the rarity and poor prognosis of hepatic MINEN tumors, multidisciplinary management is essential. Advanced molecular profiling may offer insights into tumor biology and potential therapeutic targets. Future research should explore novel systemic therapies, including immune checkpoint inhibitors, to improve patient outcomes.
背景:肝脏混合性神经内分泌-非神经内分泌肿瘤(MINEN)极为罕见,其临床行为、发病机制及最佳治疗方案的相关数据有限。肝细胞癌(HCC)与神经内分泌癌(NEC)在肝脏中共存,给诊断和治疗带来了挑战。方法:在PubMed上进行了系统性文献检索,识别肝脏原发性混合性HCC和NEC病例。检索遵循PRISMA指南,并对相关研究进行了批判性分析。共回顾了45例已报道病例,重点关注患者人口统计学特征、临床表现、治疗策略及预后。结果:大多数患者(90%)为男性,中位年龄为66.5岁。74%的病例存在乙型或丙型肝炎病毒感染,38%报告有肝硬化。混合型是最常见的组织学模式(65%)。治疗方式多样,包括经动脉化疗栓塞术(TACE)、射频消融术(RFA)、手术及全身性治疗。中位总生存期为10个月,凸显了此类肿瘤的侵袭性。结论:鉴于肝脏MINEN肿瘤的罕见性和不良预后,多学科综合治疗至关重要。先进的分子谱分析可能为肿瘤生物学及潜在治疗靶点提供见解。未来研究应探索包括免疫检查点抑制剂在内的新型全身性疗法,以改善患者预后。