摘要目的 探讨结直肠神经内分泌肿瘤的临床病理特征及其与2010年版WHO消化系统肿瘤分类胃肠胰神经内分泌肿瘤命名和分级的关系.方法 复习73例结直肠神经内分泌肿瘤的临床病理资料,重新阅读所有病例的存档切片,免疫组织化学染色采用EnVision法,53例有随访结果.结果 本组病例中男41例,女32例,中位年龄53岁(19 ～ 79岁),65例肿瘤位于距肛门10 cm以内的直肠,45例肿瘤≤1 cm,无转移病例.11例肿瘤最大径1～2 cm,其中有2例转移.17例肿瘤最大径＞2 cm,其中12例转移,转移率与肿瘤大小密切相关(P=0.000).免疫组织化学标记证实所有病例均有神经内分泌标志物表达[突触素和(或)嗜铬粒素A阳性],按照2010年WHO消化系统肿瘤分类关于神经内分泌肿瘤的命名和分类标准,本组中神经内分泌瘤(NET) 65例,其中1级(NET,G1)51例,2级(NET,G2) 14例,神经内分泌癌(NEC,G3)4例,混合性腺神经内分泌癌(MANEC)4例.随访资料显示,G1获随访的34例无肿瘤相关死亡病例,2例分别转移至盆腔和肝脏,其余均无复发转移(6 ～ 179个月)；G2获随访的12例中有5例转移,其中2例肿瘤相关性死亡,存活9例(17～118个月)；4例G3均转移,其中3例肿瘤相关性死亡；混合性腺神经内分泌癌4例,有2例肿瘤相关死亡.本组肿瘤各级别之间转移率和病死率及总生存率的差异均有统计学意义(P=0.000).结论结直肠神经内分泌肿瘤是一组预后差异显著的肿瘤,大多数显示惰性的生物学行为,2010年版WHO消化系统肿瘤分类神经内分泌肿瘤命名和分级与此类肿瘤的临床生物学行为之间有较好的对应关系.

abstractsObjective To investigate the clinicopathological characteristics of colorectal neuroendocrine neoplasms (NENs) and the prognostic significance of the new WHO classification and staging system about gastroenteropancreatic NENs.Methods The clinical and pathological records were reviewed in 73 patients with colorectal NENs (carcinoids).All slides were retrieved and reviewed,immunohistochemical staining (EnVision method) was perfomed and follow-up information retrieved.Results Forty-one men and thirty-two women were included with a median age of 53 years (19-79 years).The location of the primary tumors in 65 patients was within 10 cm from the anorectal line.In 45 cases,the tumor diameter was ≤ 1 cm (no metastasis occurred) ; in 11 cases,the tumor diameter was ＞ 1 cm but ≤2 cm (two patients had metastatic tumors) ; in 17 cases,the tumor diameter was ＞ 2 cm (12 patients had metastatic tumors).The metastatic rate was significantly correlated with tumor size (P =0.000).All tumors were immunoreactivity for synaptophysin and/or chromogranin A.According to the criteria of WHO classification and staging system about gastroenteropancreatic NENs,there were 65 cases of neuroendocrine tumors,including 51 cases of grade 1 (G1),14 cases of grade 2 (G2),4 cases of neuroendocrine carcinoma (G3) and 4 cases of mixed adenoneuroendocrine careinona.Following-up data showed that of the 34 patients with Gl tumor,there were no tumor-related death,but two patients showed metastases,and the remaining patients were disease free for 6 to 179 months.Of the 12 patients with G2 tumors,five developed metastasis,there were two tumor-related deaths,and the nine surviving patients were alive for 17 to 118 months.Of the four G3 patients,all developed metastasis and there were three tumor-related deaths.Of the four mixed adenoneuroendocrine carcinoma there were two tumor-related deaths.The difference of metastatic rate,tumor-related mortality,and overall survival among different grading groups in this series was statistically significant (P =0.000).Conclusions Colorectal neuroendocrine neoplasm is a group of tumors with distinct prognostic difference,and most of these tumors show an indolent clinical behavior.There is a good correlation between the new WHO classification and staging system of gastroenteropancreatic NENs and their clinical behaviors.