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[结直肠神经内分泌肿瘤的临床病理特征及世界卫生组织分期系统的预后意义]

[Clinicopathological features of colorectal neuroendocrine neoplasms and prognostic significance of WHO staging system].

作者信息

Zhang Xin-hua, Lu Xia-liang, Wu Nan, Liu Biao, Wang Fang-yu, Zhang Ru-song, Zhou Xiao-jun

机构信息

Department of Pathology, Nanjing General Hospital of Nanjing Military Command, Nanjing, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2013 Mar;42(3):191-6. doi: 10.3760/cma.j.issn.0529-5807.2013.03.011.

Abstract

OBJECTIVE

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 performed 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 carcinoma. Following-up data showed that of the 34 patients with G1 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.

摘要

目的

探讨结直肠神经内分泌肿瘤(NENs)的临床病理特征以及世界卫生组织(WHO)关于胃肠胰神经内分泌肿瘤的新分类和分期系统的预后意义。

方法

回顾性分析73例结直肠NENs(类癌)患者的临床和病理记录。检索并复查所有切片,进行免疫组织化学染色(EnVision法)并获取随访信息。

结果

纳入41例男性和32例女性,中位年龄53岁(19 - 79岁)。65例患者的原发肿瘤位于距肛门直肠线10 cm以内。45例肿瘤直径≤1 cm(无转移);11例肿瘤直径>1 cm但≤2 cm(2例患者有转移瘤);17例肿瘤直径>2 cm(12例患者有转移瘤)。转移率与肿瘤大小显著相关(P = 0.000)。所有肿瘤突触素和/或嗜铬粒蛋白A免疫反应阳性。根据WHO关于胃肠胰神经内分泌肿瘤的分类和分期标准,有65例神经内分泌肿瘤,包括51例1级(G1)、14例2级(G2)、4例神经内分泌癌(G3)和4例混合性腺神经内分泌癌。随访数据显示,34例G1肿瘤患者无肿瘤相关死亡,但2例出现转移,其余患者无病生存6至179个月。12例G2肿瘤患者中,5例发生转移,2例肿瘤相关死亡,9例存活患者存活17至118个月。4例G3患者均发生转移,3例肿瘤相关死亡。混合性腺神经内分泌癌4例中有2例肿瘤相关死亡。本系列不同分级组间转移率、肿瘤相关死亡率和总生存率的差异有统计学意义(P = 0.000)。

结论

结直肠神经内分泌肿瘤是一组预后差异明显的肿瘤,其中大多数肿瘤临床行为惰性。WHO关于胃肠胰神经内分泌肿瘤的新分类和分期系统与其临床行为有良好的相关性。

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