摘要
目的探讨阑尾神经内分泌肿瘤（a-NEN）的临床病理特征及其与预后的关系。方法对复旦大学附属中山医院2000年1月至2016年12月间收治的20例阑尾神经内分泌肿瘤患者的临床病理资料进行回顾性病例系列研究。根据2010年WHO消化系统肿瘤分类标准进行病理诊断，并根据核分裂数及Ki-67阳性指数对肿瘤进行分级，G1、G2级合称神经内分泌瘤（NET），G3级称为神经内分泌癌（NEC），将杯状细胞类癌等特殊类型或其他混合性神经内分泌肿瘤诊断为混合性腺神经内分泌癌（MANEC）。通过电话和门诊复诊等方式跟踪患者的预后情况，采用Kaplan-Meier法计算患者生存情况，并应用log-rank检验进行单因素预后分析。
结果20例a-NEN患者中男性14例，女性6例；中位年龄54岁。17例患者表现为急性右下腹痛，1例表现为慢性右下腹痛，1例为持续性腹部不适伴突发全腹痛，1例无症状体检发现。病理分级：G1级8例，G2级4例，G3级3例，MANEC 5例。就诊时伴有肝脏转移1例，腹腔及盆腔转移1例；术后病理发现淋巴结转移2例。6例患者行单纯阑尾切除术，12例在阑尾切除术后加行右半结肠切除术；1例行小肠切除术加右半结肠切除术；1例行肝部分切除术后加行右半结肠切除术。全组随访时间为7~187（平均36）月，1、3年生存率分别为94.7%和60.2%。单因素预后分析结果显示，年龄〉 50岁（χ2= 7.036，P= 0.008）、病理分级为MANEC（χ2= 5.297，P= 0.021）及就诊时出现转移者（χ2= 6.558，P= 0.010）5年生存率相对较低。结论a-NEN无典型临床症状，患者多以急性右下腹痛就诊；50岁以上、MANEC病理类别、就诊时发现转移者预后较差。
Objective To investigate clinicopathoIogical features and prognostic factors of appendiceal neuroendocrine neoplasms （a-NEN）. Methods Clinical data of 20 patients diagnosed with a-NEN at Zhongshan Hospital of Fudan University between January 2000 and December 2016 were retrospectively analyzed. Pathological diagnosis was based on the WHO classification criteria of digestive system tumors （2010 edition）. Based on the mitotic count and Ki-67 index, a-NENs were divided into grade 1 neuroendocrine tumor （NET G1）, grade 2 （G2） NET G2） and grade 3 （neuroendocrine carcinoma, NEC）. Some special types of a-NEN （e.g. goblet cell carcinoid） and mixed adenoneuroendocrine neoplasms were classified as mixed adenoneuroendocrine carcinoma （MANEC）. Follow-up was conducted by telephone or return visits. Univariate analysis was carried out usin theKaplan-Meier method, and the log-rank test was used to draw survival curves. Results Of 20 patients, 14 were male and 6 were female with median age of 54 years. Seventeen cases presented acute fight lower quadrant abdominal pain, 1 chronic right lower quadrant abdominal pain, 1 persistent abdominal discomfort with outburst whole abdominal pain and 1 was found during body check without symptoms. Twenty eases comprised 8 GI patients, 4 G2 patients, 3 G3 patients, and 5 MANEC patients. When diagnosed, there was 1 patient with liver metastasis, 1 patient with abdominal and pelvic metastases, and 2 patients with postoperative pathological findings of lymph node metastasis. Six patients underwent appendectomy, 12 underwent right hemicolectomy, 1 underwent right hemicolectomy plus small intestine resection, and 1 underwent partial hepatectomy plus right hemicoleetomy. The follow-up time was 7-187 months （average, 36 months）. The total 1- and 3-year survival rates were 94.7% and 60.2%, respectively. Univariate analysis showed that age 〉 50 years （X2= 7.036, P= 0.008）, pathology grade as MANEC （X2= 5.297, P= 0.021）, and metastasis （X2= 6.558, P= 0.010�
作者
毛威麟
吕洋
浦宁
李剑昂
辛宝宝
陈文祺
靳大勇
楼文晖
许雪峰
Mao Weilin;Lyu Yang;Pu Ning;Li Jian＇ ang;Xin Boobao;Chen Wenqi;Jin Dayong;Lou Wenhui;Xu Xuefeng(Department of General Surgery, Zhongshan Hospital, Fudan University, Shanghai 200032, China （ Mao WL, Lyu Y, Pu N, Li JA, Chen WQ, Jin DY, Lou WH, Xt~ XF;Department of General Surgery, Jinshan Hospital, Fudan University, Shanghai 201508, China （ Xin BB)
出处
《中华胃肠外科杂志》
CAS
CSCD
北大核心
2018年第5期564-568,共5页
Chinese Journal of Gastrointestinal Surgery
基金
国家自然科学基金（81572294）
关键词
神经内分泌肿瘤
阑尾
外科手术
预后
Neuroendocrine neoplasms
Appendix
Surgical procedures
Prognosis
