肝原发性神经内分泌肿瘤6例临床病理分析

Primary hepatic neuroendocrine neoplasms: a clinicopathological analysis of 6 cases

目的 分析肝原发性神经内分泌肿瘤（PHNEN）的临床病理学特点,探讨其相关的诊断、鉴别诊断、治疗与预后.方法 分析6例肝原发性神经内分泌肿瘤患者的临床病史、影像学、组织学形态特点及免疫组化标记结果,并复习相关文献.结果 6例中5例主诉为腹部胀痛不适,2例肝轻度肿大、有触痛.腹部彩超、CT均提示多发性肝占位.4例提示肝细胞癌或转移性癌;2例提示肝囊肿.镜下见肿瘤细胞呈巢状、小梁状或菊形团样,瘤细胞形态多为单一性,血窦丰富.免疫组化示Syn、CgA、CD56、ckpan和villin（＋）,AFP、HepPar1、glypican-3、TTF1、CDX2、CK20和CK7均（-）,Ki-67增殖指数为2％～15％.2例手术切除肿瘤,4例行介入栓塞化疗并单纯化疗.随访6例患者,1例死亡,5例状况良好.结论 肝原发性神经内分泌肿瘤是一种进展相对缓慢、罕见的恶性肿瘤,腹痛、腹胀是其共同的临床特征,影像学常提示为多发性占位,确诊依靠组织形态学和免疫组化标记.手术完整切除加术后辅助化疗是主要的治疗手段,预后取决于肿瘤的病理特征、诊疗时机和治疗方法.

Objective To analyze the clinicopathologic features of primary hepatic neuroendocrine neoplasm （PHNEN） and to discuss the diagnosis,differential diagnosis,therapy and prognosis.Methods Six cases of PHNEN were analyzed by clinical history,image features,HE and immunohistochemitry,and the related literatures were reviewed.Results The first symptom was abdominal swelling in all patients and mild swelling and tenderness of liver in 2 patients.All patients were found liver multiple masses by CT and color Doppler ultrasound,4 of 6 cases were considered as hepatocellular carcinoma or metastatic carcinoma,and 2 of 6 cases as liver cysts.The features of histopathology included the tumor cells were arranged in nests,trabeculae or rosettes.The cells were almost single morphology and blood sinus was rich in the tumors.The positive stainings of Syn,CgA,CD56,Ckpan and villin supported the diagnosis of neuroendocine tumor by immunohistochemitry.AFP,Hep Par1,Glypican-3,TTF1,CDX2,CK7 and CK20 were negative stainings.The positive ratio of Ki-67 was between 2％ and 15％.The 2 cases underwent surgical removal of the tumors and 4 cases underwent interventional embolism chemotherapy and/or chemotherapy alone.1 case died and 5 cases survived in the follow-up.Conclusions PHNEN is a rare and slowly progressing malignant tumor.The common clinical feature is abdominal pain and swells.The image features are multiple masses.Its diagnosis is depended on the features of histology and immunohistochemistry.The main treatment is complete resection combined with adjuvant chemotherapy after operation.The prognosis is associated with pathological grade,time of the diagnosis and therapeutic method of the tumor.