探讨胰腺神经内分泌肿瘤（pNENs）肝转移的临床病理特征、治疗方法及预后。

回顾性分析2019年10月天津市第一中心医院收治的1例pNENs肝转移患者临床资料。患者男，25岁，因"间断腹痛3年，皮肤巩膜黄染1年"入院。体检：皮肤、巩膜重度黄染，全腹无压痛、反跳痛及肌紧张，移动性浊音阳性。实验室检测：Hb 90 g/L，ALB 33 g/L，ALT 73 U/L，AST 88 U/L，TB 343 μmol/L，DB 279 μmol/L，ALP 1 361 U/L，GGT 743 U/L，CA19-9 128 kU/L，神经元烯醇化酶（NSE）19.1 μg/L。腹部CT检查示胰头肿物伴肝内多发结节。PET-CT检查示：胰头部不规则软组织密度结节，肝多发低密度灶。胰头及肝内占位穿刺活检病理示pNENs。诊断为pNENs肝转移。患者签署知情同意书，符合医学伦理学规定。经积极术前准备，于2020年1月7日行肝胰联合移植术。

术后病理示pNENs肝转移，G2级，免疫组化示CgA（+），Syn（+），Ki-67指数约10%。采用巴利昔单抗+甲泼尼龙免疫诱导，予他克莫司+吗替麦考酚酯+肾上腺皮质激素预防排斥反应。移植肝脏、移植胰腺功能恢复顺利。随访至术后13个月，PET-CT检查见左锁骨区、心包旁、胰腺后方、腹主动脉周围多发淋巴结转移，有复发征象，移植肝脏、移植胰腺功能正常。采用醋酸奥曲肽微球持续治疗，目前带瘤存活。

pNENs肝转移缺乏特异性表现，常表现为腹痛、黄疸，CA19-9、NSE升高和免疫组化指标CgA、Syn阳性有助于诊断。对于合并广泛肝转移的患者，肝胰联合移植可改善患者的生活质量。

To investigate the clinicopathological features, treatments and prognosis of liver metastases from pancreatic neuroendocrine neoplasms (pNENs).

Clinical data of 1 patient with liver metastases from pNENs admitted to Tianjin First Central Hospital in October 2019 were retrospectively analyzed. The 25-year-old male patient was admitted to hospital due to "intermittent abdominal pain for 3 years and yellow coloration of the skin and sclera for 1 year". Physical examination found severe yellow coloration of the skin and sclera, no tenderness, rebound pain or muscle tension of the whole abdomen, and the shifting dullness was positive. Laboratory test results showed that Hb was 90 g/L, ALB 33 g/L, ALT 73 U/L, AST 88 U/L, TB 343 μmol/L, DB 279 μmol/L, ALP 1 361 U/L, GGT 743 U/L, CA19-9 128 kU/L and neuron-specific enolase (NSE) 19.1 μg/L. Abdominal CT scan indicated a pancreatic head tumor with multiple nodules in the liver. PET-CT revealed irregular nodules with soft-tissue density in the pancreatic head complicated with multiple low-density liver metastases. The pathological findings of biopsy of pancreatic head and liver space-occupying lesions indicated pNENs. He was diagnosed with liver metastases from pNENs. The informed consent of this patient was obtained and the local ethical committee approval was received. After adequate preoperative preparations, combined liver-pancreas transplantation was performed on January 7, 2020.

Postoperative pathological examination showed intermediate-grade (G2) liver metastases from pNENs. Immunohistochemistry showed CgA (+), Syn (+), and Ki-67 index was approximately 10%. Immune induction therapy baliximab + methylprednisolone was given. Tacrolimus +mycophenolate mofetil + adrenocortical hormone were delivered to prevent rejection. The function of liver and pancreas grafts was properly recovered. During 13-month follow-up, multiple lymph node metastases in the left clavicle, pericardium, posterior pancreas and abdominal aorta were found by PET-CT. The signs of recurrence were noted. The function of liver and pancreas grafts was normal. The patient was treated with octreotide acetate-loaded microspheres consecutively and currently survived with tumors.

No specific manifestations can be found for liver metastases from pNENs, but abdominal pain and jaundice can be observed in most case. Elevated CA19-9 and NSE levels and positive CgA and Syn contribute to clinical diagnosis. Combined liver-pancreas transplantation can improve the quality of life of patients complicated with extensive liver metastases.