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目的总结原发性肝脏神经内分泌癌(primary hepatic neuroendocrine carcinoma,PHNEC)的临床及影像学特征。方法回顾性分析5例经组织病理证实PHNEC患者的临床资料。结果临床表现为疲劳、腹痛、腹部肿块;彩超示病灶呈病灶呈高回声2例,低回声1例,混合回声2例;CT显示病灶呈高密度影2例,低密度影1例,低密度影且有卫星灶2例;2例行MRI显示病灶在T1WI呈低信号,T2WI呈高信号;肿瘤位于左半肝1例,肝左外叶1例,右半肝1例,多发病灶2例;肿瘤直径3.0~12.3cm;组织病理示低分化癌2例,中分化癌1例,高分化癌2例;免疫组织化学检查示5例患者突触素、嗜铬素A、神经元特异性烯醇化酶均阳性;5例患者均行手术治疗,术后组织病理诊断为PHNEC;5例患者均存活,无瘤生存时间3~42个月。结论 PHNEC缺乏典型的临床症状和影像学表现,免疫组织化学检查可确诊,治疗首选手术切除。
Objective To summarize the clinical and imaging features of primary hepatic neuroendocrine carcinoma (PHNEC). Methods The clinical data of 5 patients with histologically confirmed PHNEC were retrospectively analyzed. The results of the clinical manifestations of fatigue, abdominal pain, abdominal mass; color Doppler ultrasound showed lesions were hyperechoic lesions in 2 cases, 1 case of hypoechoic mixed echo in 2 cases; CT showed lesions were high density in 2 cases, 1 case of low density, low density There were 2 cases of satellite lesions and 2 cases of MRI. The lesions showed low signal on T1WI and high signal on T2WI in 2 cases. The tumor located in 1 case of left liver, 1 case of left liver and 1 case of right liver, 2 cases of multiple lesions ; Tumor diameter 3.0 ~ 12.3cm; histopathology showed poorly differentiated carcinoma in 2 cases, moderately differentiated carcinoma in 1 case, well-differentiated carcinoma in 2 cases; immunohistochemistry showed 5 cases of patients with synaptophysin, chromogranin A, neuron-specific Enolase were positive; 5 patients underwent surgical treatment, postoperative histopathological diagnosis of PHNEC; 5 patients were alive, tumor-free survival time of 3 to 42 months. Conclusion PHNEC lacks the typical clinical symptoms and imaging findings. Immunohistochemical examination can confirm the diagnosis and treatment is the first choice of surgical resection.