混合性肝细胞肝癌和神经内分泌癌5例临床病理分析

Mixed hepatocellular carcinoma and neuroendocrine carcinoma:clinicopathological analyses of five cases

目的探讨混合性肝细胞肝癌和神经内分泌癌(mixed hepatocellular carcinoma and neuroendocrine carcinoma,mHNEC)的临床病理学特征。方法回顾性分析5例mHNEC的临床病理学及免疫表型特征并复习相关文献。结果5例患者均为男性,年龄25~78岁,平均59岁,3例因腹痛、腹胀入院,4例有乙肝病史。眼观:mHNEC中肝细胞肝癌(hepatocellular carcinoma,HCC)成分灰黄色,质软;神经内分泌癌(neuroendocrine carcinoma,NEC)成分灰白、灰红色,可伴坏死或出血。镜检:mHNEC中HCC成分和NEC成分可呈碰撞或混合穿插生长,其中HCC呈细梁、粗梁状排列,肿瘤细胞胞质丰富,核圆形,核仁明显;NEC呈巢、团状生长,核呈卵圆形,核质比高,核分裂象易见。免疫表型:mHNEC中HCC成分Hep Par1阳性,CD56、CgA和Syn均阴性;NEC成分Hep Par1阴性,CD56、CgA和Syn均阳性。NEC成分Ki-67增殖指数高于HCC成分。5例均行部分肝切除术,4例术后辅以综合治疗。术后随访1~49个月,2例死亡。结论肝脏mHNEC少见,其临床表现缺乏特异性,确诊依赖于病理特征及免疫表型。治疗方式主要是手术切除联合化疗。

Purpose To investigate the clinicopathological characteristics of mixed hepatocellular carcinoma and neuroendocrine carcinoma(mHNEC).Methods The clinical,morphological,and immunohistochemical features of five patients diagnosed as mHNEC were retrospectively evaluated,and relevant literatures were reviewed.Results The age of 5 patients ranged from 25 to 78(mean 59)years and four patients had hepatitis B.Three patients went to hospital because of abdominal pain and/or abdominal distention.Macroscopically,hepatocellular carcinoma component was soft yellow,neuroendocrine carcinoma component was greyish-white and showed foci of necrosis and/or haemorrhage.Microscopically,it could present as a collision tumor that showed two histologically separate parts or it could present as a combined tumor in which both types of tumor intermingled with each other.HCC component showed microtrabecullar or macrotrabecullar growth pattern,the tumor cells were moderately differentiated with pale cytoplasm and positive for Hep Par1.The NEC component was the compact growth of round cells with a high nuclear/cytoplasmic(N/C)ratio,these tumor cells were positive for neuroendocrine markers,such as Syn,CgA and CD56.The Ki-67-positive proliferating cell rate was markedly higher in the NEC component than that in the HCC component.Four patients underwent surgery and comprehensive therapy,including transarterial chemoembolization(TACE)and chemotherapy.Follow-up was obtained in 4 patients and follow-up period was 1 to 49 months.Two patients died.Conclusion mHNEC is rare with no specific symptoms.Diagnosis is based on both histological and immunohistochemical examinations.Aggressive chemotherapy is necessary after surgery removal.