摘要目的：探讨胃神经内分泌肿瘤（NEN）患者的临床分型及各型患者的临床病理特点，为临床诊治提供参考。方法回顾性总结2011年1月至2016年6月期间在北京中日友好医院和广州中山大学第一附属医院（分别为174例和67例）就诊的共计241例胃神经内分泌肿瘤患者的临床病理资料。根据胃镜表现、血清胃泌素以及胃酸监测等检查结果，将胃NEN患者分为Ⅰ～Ⅳ型，Ⅰ型为血清胃泌素升高伴胃酸分泌减少；Ⅱ型为血清胃泌素升高伴胃酸分泌增多；Ⅲ型为血清胃泌素和胃酸分泌均正常；Ⅳ型为低分化胃神经内分泌癌（NEC），病理分级为G3级或混合性腺神经内分泌癌（MANEC）。对各型患者的临床病理特点及治疗预后进行分析。结果全组241例患者中，Ⅰ、Ⅱ、Ⅲ、Ⅳ型分别为86例（35.7％）、7例（2.9％）、61例（25.3％）和87例（36.1％）。Ⅰ型86例患者中，73例（84.9％）为多发病灶，66例（76.7％）肿瘤＜1 cm，均为息肉样或颗粒状；2例（2.3％）出现转移，69例（80.2％）病理分级为G1级神经内分泌瘤（NET）；分别采用内镜下治疗（占79.1％，68／86）及外科手术切除（占11.6％，10／86），对胃内多发且内镜切除后反复复发者使用生长抑素类似物（SSA）治疗。Ⅱ型7例患者中，4例为胃泌素瘤，3例多发性内分泌腺瘤病Ⅰ型（MEN-Ⅰ）；5例出现转移；采取手术切除和SSA及质子泵抑制剂（PPI）治疗。Ⅲ型61例患者中，49例（80.3％）为单发病灶，25例（41.0％）肿瘤＞2 cm，29例（47.5％）出现淋巴结及远处转移；分别采取内镜下切除（占37.7％，23／61）、外科手术（占32.8％，20／61）和SSA药物等治疗。Ⅳ型87例患者中，74例（85.0％）肿瘤单发，51例（58.6％）肿瘤直径＞2 cm，35例（40.2％）肿瘤位于贲门部，65例（74.7％）有淋巴结转移或远处转移，采取化疗（占24.1％，21／87）或手术加化疗（占36.8％，32／87）为主。截至随访结束（2016年6月30日），全组共计死亡58例，其中Ⅰ型1例，Ⅲ型12例，Ⅳ型45例；全组总体生存率为74.2％，其中Ⅰ型98.8％，Ⅱ型均存活，Ⅲ型79.3％，Ⅳ型39.2％；Ⅲ型与Ⅳ型胃NEN总体生存率比较，差异有统计学意义（P＝0.000）。结论胃NEN临床Ⅰ～Ⅳ型分类法对其治疗和预后评估有重要意义。Ⅰ型和Ⅱ型胃NEN患者预后良好，Ⅲ型预后较差，Ⅳ型预后最差。

abstractsObjective To study subtype classification of gastric neuroendocrine neoplasm (NEN) and their clinicopathological characteristics in order to provide reference for clinical practice. Methods Clinicopathological data of 241 gastric NEN patients (174 cases from China-Japan Friendship Hospital and 67 cases from The First Affiliated Hospital of Sun Yat-Sen University ) between January 2011 and June 2016 were retrospectively summarized. According to serum gastrin , 24-hour intragastric pH monitoring and pathological grade, patients with gastric NEN were divided into 4 types: type Ⅰ(hypergastrinemia and achlorhydria, related to autoimmune chronic atrophic gastritis), type Ⅱ[hypergastrinemia and Zollinger-Ellison syndrome, related to gastrinoma or multiple endocrine neoplasia typeⅠ (MEN-Ⅰ)], typeⅢ (sporadic disease with normal serum gastrin level), and type Ⅳ[poorly differentiated gastric neuroendocrine carcinoma (NEC) and mixed adenoneuroendocrine carcinoma (MANEC)]. Clinicopathological features, treatment and prognosis of 4 types were analyzed. Results Of 241 gastric NEN cases, there were 86 cases (35.7%) in type Ⅰ, 7 cases (2.9%) in type Ⅱ, 61 cases (25.3%) in type Ⅲ and 87 cases(36.1%) in type Ⅳ. Among 86 cases of type Ⅰ gastric NEN, 73 cases (84.9%) were multiple lesions,tumor size of 66 cases (76.7%) was less than 1 cm, all the 86 cases were polypoid or granular lesions. 2 cases (2.3%)presented distant metastasis, 69 cases (80.2%) had pathological grading as NET G1; most of them received endoscopic surgery treatment and follow-up; somatostatin analogs(SSA) was used in patients with multiple lesions and repeated recurrence after endoscopic treatment. Among 7 cases of type Ⅱ, 4 cases were gastrinoma, 3 cases MEN-Ⅰ;5 cases presented distant metastasis; treatment included surgery, SSA and proton pump inhibitor (PPI) therapy. Among 61 cases of type Ⅲ gastric NEN, 49 cases(80.3%) were single lesion,tumor size of 25 cases(41.0%) was more than 2 cm, 29 cases(47.5%) had lymph node metastasis or distant metastasis;treatment included endoscopic resection, surgery or SSA therapy according to the tumor staging. Among 87 patients of type Ⅳ gastric NEN, 74 cases (85.0%) had single lesion,tumor size of 51 cases (58.6%) was more than 2 cm; lesions were found in gastric cardia in 35 cases (40.2%); 65 cases (74.7%) had lymph node metastasis or distant metastasis; treatment included chemotherapy, or surgery plus chemotherapy. At the end of follow-up (June 30, 2016), 58 patients were dead, including 1 case of typeⅠ, 12 cases of typeⅢ and 45 cases of typeⅣ. The overall survival rate of all the patients was 74.2%, and was 98.8%, 100%, 79.3%, 39.2% of types Ⅰ, Ⅱ, Ⅲ, Ⅳrespectively. The overall survival rate between typeⅢ and typeⅣ gastric NEN was significantly different (P=0.000). Conclusions Subtype classification of gastric NEN is very significant for making therapeutic decisions and prognostic evaluation. Patients of typeⅠ or typeⅡ gastric NEN have good prognosis,while those of typeⅢ and type Ⅳ have poor prognosis, and those of typeⅣ have the worst prognosis.