消化系神经内分泌癌及文献分析209例

Neuroendocrine carcinoma in digestive system:case analysis and literature review

目的:探讨消化系统神经内分泌癌(NEC)发生部位、临床表现、内镜及影像学特点、病理特征、诊断和鉴别诊断、治疗及预后．方法:将我院2000-2005年收治的7例NEC病例和文献检索的202例病例分为A、B两组,分别探讨消化系统NEC临床特点,治疗和预后．结果:消化系统NEC临床表现多为无功能NEC,以局部占位及消耗症状为主,壶腹周围NEC可以急性胰腺炎发作为首发表现:有功能的NEC例数约占2．4%．大小在0．5-13．0 cm,分为典型类癌、不典型类癌和小细胞癌,确诊需要进行突触素(Syn)、细胞角蛋白(CK)、神经特异性烯醇化酶(NSE)、嗜铬素A(CgA)、上皮膜抗原(EMA)等的免疫组化检查和(或)电镜检查发现神经内分泌颗粒(NSG)．以手术治疗为主,辅以化疗、放疗,必要时栓塞化疗,也可以辅以生物治疗．对于有功能的NEC,首先需要控制因激素分泌过多所致的相关类癌综合征．小细胞癌预后最差,不典型类癌次之,类癌预后较好,5 a生存率可达70%．肿瘤的大小、转移与否、手术切除是否彻底等等亦是影响预后的重要因素．结论:消化系统NEC发生部位以胃、胰腺、直肠等部位多见,临床表现多样,少数可有类癌综合征表现,治疗方法以手术为主,辅以放化疗及生物治疗或介入治疗,根据病理类型判断其恶性程度和预后．

AIM： To analyze the originating sites, clinical manifestations, endoscopic and imaging features, pathologic findings, treatments and prognosis of patients suffered from neuroendocrine carcinoma （NEC） in digestive system. METHODS： A total of 209 cases, of which 7 cases were from our hospital and the other 202 cases were reported in literatures from 1994 to 2005, were classified as group A and B, respectively. Clinical presentation, therapy and prognosis of NEC arising from digestive system were discussed. RESULTS： NEC mainly occurred in stomach, pancreas, rectum, esophagus, small intestine, Vater ampullae of duodenum, liver and so on, and it had a wide spectrum of clinical presentations such as abdominal pain, epigastric distention, symptoms of wasting. PeriampuUary NEC might cause （recurrent） acute pancreatitis, and most of them were non-functioning neoplasms. Only about 2.4% of NEC cases presented an endocrine syndrome. NEC was classified as typicalcarcinoid, atypical carcinoid and small cell carcinoma, and its masses varied from 0.5 to 13.0 cm in diameter, and the diagnosis of NEC depended on the features of endoscopic examination, B-ultrasound （BIDS）, computed tomography （CT）, endoscopic retrograde cholangiopancreatography （ERCP）, endoscopic ultrasonography （EIDS） and pathologic findings, especially immunohistochemical markers such as synaptophysin （Syn）, cytokeratin （CK）, neuro-specific enolase （NSE）, chromogranin A （CgA）, epithelial membrane antigen （EMA） and （or） neurosecretory granules （NSG） observed through electronic microscopy. Operation was the dominant strategy in the treatment of NEC, assisted by post-operative chemotherapy, radiotherapy, chemoembolization or biotherapy. The initial treatment of functioning NEC was to control the symptoms caused by excessive hormonal production. Typical carcinoid had a better prognosis than atyptical carcinoid or small cell carcinoma, and the 5-year survival rate was as high as 70%. Other important prognostic factors consisted of the size of the mass, metastasis, and the type of surgical resection. CONCLUSION： NEC in digestive system often originates from stomach, pancreas, rectum, etc. It has miscellaneous clinical manifestations and rare cases have carcinoid syndrome. The diagnosis of NEC mainly depends on pathologic findings and operation is the first-line treatment. The prognosis of NEC is also correlated with the pathologic classifications.