胆囊神经内分泌癌10例单中心临床分析

Neuroendocrine carcinoma of the gallbladder： ten cases in a single institution

目的 总结分析胆囊神经内分泌癌（GB-NEC）的临床特征及诊治经验.方法 回顾性分析10例GB-NEC患者资料,并与同期377例胆囊腺癌比较.结果 GB-NEC患者占同期胆囊癌患者总数的2.2％ （10/464）.患者平均年龄（59.0±10.0）岁,男女之比1∶4.10例患者均为低分化小细胞GB-NEC,4例合并腺癌.免疫组化CgA、NSE、Syn、EMA阳性率分别为100％ （10/10）、100％（10/10）、88.9％ （8/9）、87.5％ （7/8）.TNM分期Ⅱ期1例,ⅣA期2例,ⅣB期7例,N2淋巴结转移达70.0％ （7/10）,明显高于同期胆囊腺癌的34.0％（128/377,P＜0.05）.2例行根治性切除手术,8例行姑息性手术.10例GB-NEC患者1年、2年、3年生存率分别为20.0％ （2/10）、10.0％ （1/10）、0,中位生存时间为92 d.同期胆囊腺癌患者1年、3年、5年生存率分别为32.7％、21.3％、11.7％,中位生存时间为180 d.结论 GB-NEC多发于老年女性,临床表现无特异性,确诊依赖于免疫组化检查.该类肿瘤恶性程度高,早期即可发生局部浸润和淋巴转移,其预后较胆囊腺癌更差.手术治疗为其首选、主要的治疗手段,联合放化疗及TACE等多种治疗手段有助于延长患者生存时间.

Objective To summarize and analyse the clinical characteristics,diagnosis and treatment options of neuroendocrine carcinoma of gallbladder （GB-NEC）.Methods The data of 10 patients with GB-NEC treated in our hospital between December 2007 and December 2012 were retrospectively analyzed and compared with the data of 377 patients with gallbladder adenocarcinoma.Results The 10 patients with GB-NEC accounted for 2.2% （10/464） of all gallbladder cancers that were treated during the study period.The mean age was 59.0 ± 10.0 years,the ratio of male to female was 1 ∶ 4.All the 10 patients had low-differentiated small cell NEC,four patients were accompanied with an adenocarcinoma.Immunohistochemical examinations showed that the positive rates of CgA,NSE,Syn,EMA were 100% （10/10）,100% （10/10）,88.9% （8/9） and 87.5% （7/8）,respectively.One,two and seven patients had a TNM grade Ⅱ,ⅣA,and ⅣB,respectively.Seven patients （70.0%） had N2 lymphatic metastases,which was significantly higher than the percentage in patients with gallbladder adenocarcinoma （128/377,34.0% ; P 〈 0.05）.Two patients were treated with radical resection,while the other 8 patients were treated with palliative surgery.The 1-,2-,and 3-year survival rates of the patients with GB-NEC were 20.0%,10.0%,and 0,respectively.The median survival time was 92 days.In contrast,the 1-,2-,3-,and 5-year survival rates of the 377 patients with gallbladder adenocarcinoma were 32.7%,25.4%,21.3%,and 11.7% respectively,and the median survival time was 180 days.Condusions GB-NEC was found mainly in aged females.The clinical presentations were mainly non-specific,and immunohistochemical examinations were needed for a definite diagnosis.GB-NEC is highly malignant,and local invasion and lymphatic metastasis could occur early.The short-term recurrence rate was very high.The prognosis of GB-NEC was poorer than gallbladder adenocarcinoma,while surgical resection combined with radiotherapy,chemotherapy and TACE increased the survival of these patients.