BACKGROUND rimary hepatic neuroendocrine neoplasms (PHNEN) are exceedingly rare tumors with atypical clinical manifestations, accounting for less than 0.5% of all neuroendocrine tumors. Currently, there is a lack of consensus on their management, and guidelines do not recommend postoperative chemotherapy for patients with stage G1/G2 disease after curative resection. We present a case report of PHNEN, outlining its diagnostic challenges, treatment strategy, and clinical outcomes. CASE REPORT A 31-year-old man presented with jaundice and was initially diagnosed with suspected IgG4-related disease, which initially appeared to respond to steroid therapy, but manifested worsening jaundice 4 months after initial treatment. Subsequent evaluation revealed a PHNEN NET G2 with lymph node metastasis and invasion of the right hepatic artery; and involvement of the hepatic duct at the hepatic hilum, primarily the left hepatic duct. The patient underwent extended left hemi-hepatectomy with caudate lobe resection, bile duct resection, and lymphadenectomy, followed by reconstruction of the right hepatic artery. Postoperatively, the patient received adjuvant chemotherapy consisting of capecitabine (1000 mg bid D1-14) and temozolomide (200 mg qn D10-14) for 6 cycles. Currently, the patient remains disease free 43 months after treatment. CONCLUSIONS PHNEN presents diagnostic challenges due to its rarity and lack of specific markers. Surgical resection remains the cornerstone of treatment, with chemotherapy being considered in select cases with high-risk features. Further research is needed to refine treatment approaches and improve outcomes for patients with PHNEN.

Intrahepatic cholangiocarcinoma (ICC) has a high degree malignancy, and its incidence has gradually increased in recent years. Surgery is still the only curative treatment for ICC to achieve long-term survival, but its onset is hidden, and most patients have lost the opportunity of operation when they are diagnosed. ICC shows significant heterogeneity. With the development of the second generation sequencing technology, the tumor patterns of ICC, such as DNA, RNA, epigenetic, protein, microenvironment and so on, have been gradually discovered. The purpose of this article is to review the research progress of molecular typing and clinical application of ICC in recent years.

Hepatocellular carcinoma is a common malignancy of digestive system. Tumor markers are important for the early diagnosis and prognosis of hepatocellular carcinoma. Protein induced by vitamin K absence or antagonist-Ⅱ (PIVKA-Ⅱ) is of high specificity and sensitivity in hepatocellular carcinoma diagnosis. In addition, previous studies suggested that PIVKA-Ⅱ played a role in proliferation, invasion and metastasis of hepatocellular carcinoma and could be used for prognosis of hepatocellular carcinoma. This article reviewed the recent advances in the underlying biological mechanism of PIVKA-Ⅱ in hepatocellular carcinoma and concluded the value of PIVKA-Ⅱ in hepatocellular carcinoma diagnosis and prognosis.

Objective:To study the relationship between morphologic classification and prognosis of patients with intrahepatic cholangiocarcinoma (ICC).Methods:The clinicopathologic data of 125 ICC patients who underwent R 0 resection at the Ningbo Medical Center Li Huili Hospital from January 2011 to May 2019 were retrospectively analyzed. This cohort consisted of 67 males and 58 females, aged 26.0 to 82.0 (63.5±9.5) years old. Based on the resected specimens, the patients were divided into the mass forming type, periductal infiltrating type and mixed type. The survival rates and recurrence-free survival outcomes of the patients among the three types were compared, and the risk factors that influenced prognosis were analyzed. Results:Using the predetermined inclusion and exclusion criteria, 16 patients with missing data and other causes of death were excluded from the initial 125 patients. Also, as the number of the mixed type was small, these patients were excluded. Finally 62 patients in the mass forming type and 42 patients in the periductal infiltrating type were analyzed. In the mass forming group, there were 32 males and 30 females, aged (63.1±9.1) years. In the perivascular infiltration group, there were 22 males and 20 females, aged (64.1±10.2) years. The 1-, 3-, 5-year survival rates of the mass forming group were 78.1%, 33.2%, 18.9% (median survival 25 months). The 1-, 3-, 5-year survival rates of the periductal infiltrating type were 63.3%, 8.3%, 6.1% (median survival 15 months). There were statistically significant differences between the two groups ( P<0.05). The median recurrence-free survival for the mass forming and the periductal infiltrating groups were 18 and 11 months, respectively, with a statistically significant differences between them ( P<0.05). Univariate and multivariate analysis showed that TNM staging Ⅲ~Ⅳ ( HR=2.966, 95% CI: 1.549-5.679) and periductal infiltrating type ( HR=2.403, 95% CI: 1.236-4.670) were independent risk factors for survival of these patients after operations. TNM staging Ⅲ~Ⅳ ( HR=2.466, 95% CI: 1.325-4.589), low grade differentiation ( HR=0.528, 95% CI: 0.299-0.934) and periductal infiltrating type ( HR=2.432, 95% CI: 1.295-4.565) were independent risk factors for relapse-free survival of these patients ( P<0.05). Conclusions:Morphological classification was found to be an independent risk factor for prognosis of ICC patients in this study, with significantly worse long-term prognosis when compared with the mass forming type.

目的 探讨胆管下段癌行根治性胰十二指肠切除术后辅助化疗效果.方法 回顾性分析自2016年1月至2019年1月宁波市医疗中心李惠利东部院区收治的23例胆管下段癌行根治性胰十二指肠切除术患者的临床资料,根据术后是否接受化疗分为2组,其中化疗组[GEMOX方案(吉西他滨1000 mg/m2,d1,d8;奥沙利铂130 mg/m2,d1;3周1疗程)]10例和未化疗组13例,比较分析两组术后生存状况(中位生存时间、12个月生存率、累积生存率及无复发生存率).结果 化疗组随访1～30个月,5例死亡,5例存活;未化疗组随访6～28个月,5例死亡,8例存活.化疗组中位生存时间为21个月,未化疗组中位生存时间为22个月(P>0.05).化疗组和未化疗组12个月生存率分别为80％(8/10)和77％(10/13).两组累积生存率比较,差异无统计学意义(χ2=0.277,P>0.05).化疗组中位无复发生存时间为15个月,未化疗组为13个月,两组无复发生存率比较,差异无统计学意义(χ2=0.002,P>0.05).结论 以吉西他滨+奥沙利铂为主要方案的术后辅助化疗不能使胆管下段癌R0切除患者明显获益.

目的 探讨胆总管下段癌行根治性胰十二指肠切除术的可行性及短期疗效.方法 回顾性分析收治并实施根治性胰十二指肠切除术的23例胆总管下段癌患者的临床资料.结果 23例患者均手术成功,平均手术时间(345.3±78.3)min,平均术中出血量(384.8±180.6)ml,平均术后住院时间(23.0±12.8)d.术后30 d内无死亡病例.术后随访1～30个月,平均无复发生存时间13.4个月,中位生存时间21个月,术后6个月及12个月生存率分别为913％(21/23)和82.6％(19/23).结论 在较大的肝胆胰外科中心,胆总管下段癌行根治性胰十二指肠切除术,安全有效,术后并发症少,短期存活率高.

The incidence of intrahepatic cholangiocarcinoma(ICC)has been increasing year by year.For most patients,surgical resection is not suitable when they are diagnosed as ICC.Conventional chemotherapy and radiotherapy are not effective for the long-term survival rate of ICC patients and lead to the poor overall prognosis.In recent years,with the deepening understanding about the molecular mechanism of biliary malignant tumors,some key genes and signaling pathways related to the pathogenesis of ICC have been identified,providing new ideas for the targeted therapy.In this paper,major molecular mechanisms and targeted therapies of ICC are reviewed.