总结原发性肝脏神经内分泌肿瘤（PHNEN）的临床表现、影像学表现、病理学特征、诊治及生存状况。

回顾性分析2020年1月至2022年1月昆明医科大学第二附属医院收治的12例PHNEN患者临床资料。其中男5例，女7例；年龄24~77岁，中位年龄66岁。患者均签署知情同意书，符合医学伦理学规定。根据2019版消化系统肿瘤WHO分类分级标准分型，分析患者临床表现、影像学表现、病理学特征、诊治情况、生存状况。

腹痛为患者主要临床表现，其中腹痛8例，2例表现为腹部包块。肿瘤单发4例，多发8例。神经内分泌瘤（NET）7例，其中G2级6例，G3级1例；神经内分泌癌（NEC）5例，其中大细胞型3例，分型不清2例。超声造影检查动脉期表现为整体高增强发生率3/5，增强CT边缘环状强化发生率6/12。MRI增强扫描动脉期强化发生率9/12。神经无特异性烯醇化酶阳性率6/10，铁蛋白阳性率5/9，CA19-9阳性率5/12。免疫组化法染色Syn阳性率11/12，CD56阳性率9/12，CgA阳性率2/12，CD34阳性率10/10，CK-19阳性率7/9。5例行手术治疗，1例行射频消融+化疗，1例行TACE治疗，1例行化疗+放疗治疗，1例行依维莫司+奥曲肽治疗，3例行穿刺活检后放弃治疗。随访时间1.0~22.0个月，中位随访时间7.5个月，随访期间7例存活，多为手术、射频消融、TACE积极治疗患者；5例死亡，多为放弃治疗患者。

PHNEN临床症状无特异性，有一定影像学检查特征，确诊依靠病理学检查。积极手术切除，联合TACE、化疗及靶向治疗可改善患者预后。

To summarize clinical manifestations, imaging finding and pathological characteristics, diagnosis, treatment and survival of primary hepatic neuroendocrine neoplasm (PHNEN).

Clinical data of 12 patients with PHNEN admitted to the Second Affiliated Hospital of Kunming Medical University from January 2020 to January 2022 were retrospectively analyzed. Among them, 5 patients were male and 7 female, aged from 24 to 77 years, with a median age of 66 years. The informed consents of all patients were obtained and the local ethical committee approval was received. According to the WHO classification of digestive system tumors in 2019, clinical manifestations, imaging finding and pathological characteristics, diagnosis and treatment, and survival of PHNEN patients were analyzed.

Abdominal pain was the main clinical manifestation, including 8 cases of abdominal pain and 2 cases of abdominal mass. 4 patients were diagnosed with single PHNEN and 8 cases of multiple PHNEN.7 cases were diagnosed with neuroendocrine tumor (NET), including 6 cases of grade G2 and 1 case of grade G3. 5 cases developed neuroendocrine carcinoma (NEC), including 3 cases of large cell type and2 cases of unknown type. Contrast-enhanced ultrasound showed that the incidence of hyper-enhancement in the arterial phase was 3/5, and the incidence of circumferential enhancement in enhanced CT scan was 6/12. Enhanced MRI indicated that the incidence of enhancement in the arterial phase was 9/12. The positive rates of neuron-specific enolase, ferritin and CA19-9 were 6/10, 5/9 and 5/12, respectively. Immunohistochemical staining showed that the positive rates of Syn, CD56, CgA, CD34 and CK-19 were 11/12, 9/12, 2/12, 10/10 and 7/9, respectively. 5 patients underwent surgery, 1 case of radiofrequency ablation +chemotherapy, 1 case of TACE, 1 case of chemotherapy + radiotherapy, 1 case of everolimus + octreotide, and 3 cases rejected further treatment after puncture biopsy. The follow-up time was ranged from 1.0 to22.0 months, with a median of 7.5 months. During subsequent follow-up, 7 patients survived, and most of them underwent surgery, radiofrequency ablation and TACE. 5 patients died, and most of them rejected treatment.

PHNEN is manifested with non-specific clinical symptoms and certain imaging features. The diagnosis of PHNEN depends on pathological examination. Active surgical resection combined with TACE, chemotherapy and targeted therapy can improve the clinical prognosis of PHNEN patients.