原发性肝脏神经内分泌肿瘤诊治经验

Diagnosis and treatment experience of primary hepatic neuroendocrine neoplasm

目的探讨原发性肝脏神经内分泌肿瘤(NEN)的诊治及疗效。方法回顾性分析2013年3月至2015年12月湖南省人民医院收治的10例原发性肝脏NEN患者临床资料。患者均签署知情同意书,符合医学伦理学规定。其中男3例,女7例;年龄21~65岁,中位年龄45岁。根据2010版消化系统肿瘤WHO分类标准,神经内分泌瘤(NET)G2 6例,神经内分泌癌(NEC)4例。分析患者临床资料、影像学特点、治疗情况、肿瘤病理学特点及预后。结果患者右上腹痛5例,右上腹包块1例,乏力、纳差1例,其余3例患者由常规体检发现。8例影像学诊断为肝孤立性肿块,肝内多发占位2例。10例患者均经病理学检查诊断,其中3例术前活检确诊,7例术后病理学检查确诊。根治性切除6例,微波消融术1例,肝脏肿块切除及静脉化疗泵植入术1例,单纯行化疗1例,放弃治疗1例。截止至投稿日期无复发生存6例;1例术后1个月复发并腹膜后多发淋巴结转移,仍存活;死亡2例,其中1例穿刺活检后放弃治疗死亡,1例化疗1周期后死于肿瘤多发远处转移;失访1例。结论肝脏NEN无特异性临床表现,需病理学检查确诊,手术切除为首选治疗方法,对于肿块不可切除的患者可选择TACE、微波消融、化疗等方法。

Objective To investigate the diagnosis, treatments for primary hepatic neuroendocrine neoplasm （NEN） and the efficacy. Methods Clinical data of 10 patients with primary hepatic NEN admitted in Hunan People＇s Hospital from March 2013 to December 2015 were analyzed retrospectively. The informed consents of all patients were obtained and the local ethical committee approval was received. There were 3 males and 7 females, aged 21-65 years old and the classification for digestive system tumor 2010 version, and 4 cases of neuroendocrine carcinoma （NEC）. The median age was 45 years old. According to the WHO there were 6 cases of neuroendocrine tumor （NET） G2, clinical data, imaging characteristics, treatment, tumor pathological characteristics and prognosis of the patients were analyzed. Results Right upper abdominal pain was observed in 5 cases, right upper abdominal mass in 1 case, asthenia and anorexial in 1 case, and the remaining 3 cases were found with tumor when receiving routine physical examination. For imaging finding, 8 cases were diagnosed as isolated liver mass, and 2 were found with multiple masses in the liver. All of the 10 patients were confirmed by pathological examination, of which 3 were diagnosed by biopsy before operation, and 7 were confirmed with pathology after operation. 6 cases underwent radical resection, 1 case received microwave ablation, 1 underwent liver resection and intravenous chemotherapy pump implantation, 1 received chemotherapy alone and 1 abandoned treatment. To the submission date of this article, 6 cases survived without recurrence, 1 case suffered recurrence 1 month after operation with multiple lymph node metastases in the peritoneum and remained alive. 2 cases died, 1 of which died of no treatment after biopsy, 1 died of multiple distant metastases after 1 period of chemotherapy, and 1 lost follow-up. Conclusions There is no specific clinical manifestations of hepatic NEN. It should be confirmed by pathological examination. Surgical resection is the first choice for treatment. For patients with unresectable mass, TACE, microwave ablation and chemotherapy can be applied.