纤维板层型肝细胞癌5例临床病理分析

Fibrolamellar hepatocellular carcinoma:a clinicopathologic study of five cases and review of literature

目的探讨5例纤维板层型肝细胞癌(FL-HCC)临床病理学特征、诊断、鉴别诊断与预后。方法收集中国人民解放军总医院第一医学中心2005—2019年诊断的5例FL-HCC,进行病理分析,结合文献并复习。结果女性3例,男性2例,年龄21~69岁,中位年龄44岁;影像学检查5例均显示肝占位性病变,其中2例MRI检查显示中央见星条状低信号瘢痕区,增强扫描病灶边缘呈明显强化,中央瘢痕区未见强化,1例显示肝右叶巨大多血供肿块,中央见低信号区伴坏死及钙化,肝左内叶异常多血供小结节,肝门及腹膜后多发肿大淋巴结。大体检查,5例肿瘤呈结节状,切面棕黄色,中央见放射状瘢痕及褐色坏死区,并见钙化;5例组织学均表现为大、多角形肿瘤细胞呈条索状或实性巢团分布,胞质嗜酸性粗颗粒状,内见嗜酸性小体及苍白小体,胞核大,泡状核,见核仁,瘤细胞巢间见板层状纤维组织,周边见内陷的胆管,中央均见纤维瘢痕及坏死。免疫组化染色示肿瘤细胞5例CK7(+),4例CD68部分点状(+),Ki-67增殖指数5%~35%;特殊染色显示5例嗜酸性小体PAS阳性,Masson染色及网织纤维染色纤维组织阳性。3例患者随访63、31、14个月仍健在,1例巨大肿瘤伴有肝门、腹腔淋巴结转移的患者20个月后死亡,1例患者术后复发3次,随访36个月失访。结论FL-HCC是一种罕见特殊类型的肝癌,发病年龄较年轻,多不合并肝炎,确诊依据病理形态及免疫组化特点。手术治疗是首选的治疗方法,手术不能切除的患者可行介入治疗辅以化疗和肝脏移植。

Objective To study the clinicopathological features,differential diagnoses and prognosis of fibrolamellar hepatocellular carcinoma(FL-HCC).Methods Five cases of FL-HCC were analyzed by clinical pathological characteristics,and related literature was reviewed.Results Three female and 2 male patients were included,aged 21 to 69 years,with median age of 44 years.MRI showed low-density lesion and a central scar was found,obvious enhancement at the edge of the lesion,but no enhancement was observed in the central striated scar area after enhancement.On gross examination,the tumor was nodular with brown-yellow section,and central radiated scar and brown necrotic area were observed.Histologically,the tumor cells were large and polygonal with cord-like or solid nest-like arrangement,and the cytoplasm of tumor cells was eosinophilic and coarsely granular.The nuclei of tumor cells were large and vesicular,with visible nucleoli;furthermore,the lamellar fiberous tissues arround the tumor cell nests were noted with biliary ducts in the periphery.All the cases of tumor cells were typically positive for CK7;CD68 showed granular dotlike cytoplasmic staining in four cases,The index of Ki-67 was 5%-35%.The eosinophilic bodies were PAS positive.Rich reticular fibers were seen in all cases.Three patients were followed up for 63 months,31 months,14 months,respectively,and were still alive.One patient with large tumor accompanied by hilar and peritoneal lymph node metastasis died after 20 months,one patient had 3 postoperative recurrences and was lost to follow-up after 3 years.Conclusion FL-HCC is a rare type of hepatocellular carcinoma,typically affects young adults and has no history of hepatitis;clinical diagnosis should be based on pathological examination.The most effective treatment for FL-HCC is surgical resection.Interventional therapy with chemotherapy and liver transplantation is feasible for patients who cannot be resected.