CAJ | 학술논문

目的探讨胃肠胰神经内分泌肿瘤(GEP-NEN)发生部位、临床症状、内镜及影像学表现、病理特点、诊断、治疗及预后。方法收集中山大学附属第一医院2000年1月至2010年6月间收治的87例GEP-NEN患者病历资料，通过对神经内分泌标记物突触素(Syn)和铬粒素A(CgA)的免疫组织化学染色确定肿瘤是否具有神经内分泌性质，按组织学和增殖活性明确肿瘤分级，将GEP-NEN分为神经内分泌瘤（G1和G2级）、神经内分泌癌（G3级）和混合性腺神经内分泌癌(G3级)，探讨其临床特点及诊疗情况。结果 36例(41.4 ％)GEP-NEN发生在胰腺，其次为直肠18例(20.7％)、胃9例(10.3％)、十二指肠6例(6.9％)。87例GEP-NEN中65例(74.7％)为非功能性，多以各种消化道症状或肿瘤局部占位为首发症状，无一例出现类癌综合征，内镜及影像学表现主要为肿瘤占位病变。87例GEP-NEN中69例(79.7％)为神经内分泌瘤(NET)、13例(14.9％)为神经内分泌癌(NEC)、5例(5.4％)为混合性腺神经内分泌癌(MANEC)；G1、G2和G3级肿瘤分别占64.9％、14.9％和20.2％。22例(47.8％) GEP-NEN患者就诊时肿瘤浸润肌层/浆膜层，18例(20.7％)出现淋巴结转移，18例(20.7％)出现远处转移，大部分转移至肝脏。CgA和Syn免疫组织化学染色阳性率分别为74.2％和88.1％。79例(90.8％)患者进行手术治疗。随访1年、3年和5年生存率分别为76.9％、54.2％和41.7％。结论GEP-NEN可发生于消化系统任何部位，临床表现多样，内镜和影像学检查是重要的诊断手段，确诊主要依赖病理，手术是主要的治疗手段，肿瘤病理分类、分级和远处转移情况等与其预后有关。
목적 탐토위장이신경내분비종류(GEP-NEN)발생부위、림상증상、내경급영상학표현、병리특점、진단、치료급예후。방법수집중산대학부속제일의원2000년1월지2010년6월간수치적87례GEP-NEN환자병력자료，통과대신경내분비표기물돌촉소(Syn)화락립소A(CgA)적면역조직화학염색학정종류시부구유신경내분비성질，안조직학화증식활성명학종류분급，장GEP-NEN분위신경내분비류（G1화G2급）、신경내분비암（G3급）화혼합성선신경내분비암(G3급)，탐토기림상특점급진료정황。결과 36례(41.4 ％)GEP-NEN발생재이선，기차위직장18례(20.7％)、위9례(10.3％)、십이지장6례(6.9％)。87례GEP-NEN중65례(74.7％)위비공능성，다이각충소화도증상혹종류국부점위위수발증상，무일례출현유암종합정，내경급영상학표현주요위종류점위병변。87례GEP-NEN중69례(79.7％)위신경내분비류(NET)、13례(14.9％)위신경내분비암(NEC)、5례(5.4％)위혼합성선신경내분비암(MANEC)；G1、G2화G3급종류분별점64.9％、14.9％화20.2％。22례(47.8％) GEP-NEN환자취진시종류침윤기층/장막층，18례(20.7％)출현림파결전이，18례(20.7％)출현원처전이，대부분전이지간장。CgA화Syn면역조직화학염색양성솔분별위74.2％화88.1％。79례(90.8％)환자진행수술치료。수방1년、3년화5년생존솔분별위76.9％、54.2％화41.7％。결론GEP-NEN가발생우소화계통임하부위，림상표현다양，내경화영상학검사시중요적진단수단，학진주요의뢰병리，수술시주요적치료수단，종류병리분류、분급화원처전이정황등여기예후유관。
Objective To explore the occurrence site, clinical manifestations, endoscopic and radiographic features, pathologic characteristics, diagnosis, treatment and prognosis of gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN).Methods From January 2000 to June 2010,medical records of 87 GEP-NEN patients in the First Affiliated Hospital of Sun Yat-sen University were collected. The neuroendocrine properties of the tumors were determined by immunohistochemistry staining of neuroendocrine markers Syn and CgA.Then according to histology and proliferation activity, the GEP-NEN tumors were classified as neuroendocrine tumor (G1 and G2 grade), neuroendocrine carcinoma (G3 grade) and mixed adenoendocrine carcinoma (G3 grade).Results 36 cases (41.4％) of GEP-NEN tumors were in pancreas, followed by 18 cases in rectum (20.7％), 9 cases in stomach (10.3％) and 6 cases in duodenum (6.9％).Total 69 of 87 GEP-NEN cases (74.7％) were nonfunctional tumors, a variety of gastrointestinal symptoms or symptoms caused by local tumor compression were the most first symptoms, and no one case presented carcinoid syndrome.The manifestations of endoscopy and radiographic image were tumor occupying lesion.Of 87 GEP-NEN cases, 69 cases were neuroendocrine tumor (79.7％), 13 cases were neuroendocrine carcinoma (14.9％) and 5 cases were mixed adenoendocrine carcinoma (5.4％).The percentage of grade G1, G2 and G3 tumors were 64.9％、 14.9％ and 20.2％ respectively.Muscularis or serosa infiltration were found in 22 GEP-NEN patients (47.8％) at the first visit, lymphatic metastasis was in 18 cases (20.7％) and distant metastasis were in 18 cases (20.7％), mostly migrated to liver.Immunohistochemistry staining positive rates of CgA and Syn were 74.2％ and 88.1％.A total of 79 patients (90.8％) were underwent surgery.The 1-, 3-and 5-year survival rates were 76.9％, 54.2％and 41.7％ during follow-up.Conclusions GEP-NEN may occur at any part of the digestive system,the clinical manifestations are variable.Endoscopy and radiographic examination play an important role in tumor diagnosis, but final diagnosis is mainly based on pathological detection, and surgery is the main treatment option.The prognosis of the tumor is correlated with the pathological classification,grading and condition of distant metastasis.