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[结直肠神经内分泌肿瘤的临床病理特征]

[Clinicopathologic characteristics of colorectal neuroendocrine tumor].

作者信息

Jung Sang Hun, Kim Hee Cheol, Yu Chang Sik, Chang Heung Moon, Ryu Min Hee, Lee Jae Lyun, Kim Jung-Sun, Kim Jin Cheon

机构信息

Department of Surgery, University of Ulsan College of Medicine, Songpa-gu, Seoul, Korea.

出版信息

Korean J Gastroenterol. 2006 Aug;48(2):97-103.

PMID:16929153
Abstract

BACKGROUND/AIMS: Colorectal neuroendocrine carcinoma is a rare neoplasm exhibiting fulminant progression and having poor prognosis. The purpose of this study is to verify the clinicopathologic characteristics of colorectal neuroendocrine carcinoma.

METHODS

From June 1997 to December 2004 at Asan Medical Center, ten patients were originally identified as colorectal neuroendocrine carcinoma on the basis of H&E and immunohistochemical staining (IHC). Carcinoid tumors were excluded in this study. Medical records of thirteen patients were reviewed retrospectively.

RESULTS

Ten patients (0.2%) with colorectal neuroendocrine tumors were identified from 4,512 patients with colorectal cancer; ten neuroendocrine carcinomas and three adenocarcinomas with neuroendocrine differentiation. Their median age was 60 (41-83) years. The subjects consisted of six males and seven females. Nine tumors were located in the rectum, two in the sigmoid, and each one in the transverse colon and cecum, respectively. Nine of ten neuroendocrine carcinomas expressed synaptophysin, but chromogranin A were expressed in four. All patients were advanced at the time of diagnosis, with AJCC TNM staging: stage IIIB (n=2), stage IIIC (n=3), and stage IV (n=8). The median survival for ten neuroendocrine carcinomas and three adenocarcinomas with neuroendocrine differentiation were 16.4 months and 30 months, respectively. Five patients who received chemotherapy showed median survival of 32 months (stage III) and 17.5 months (stage IV), whereas other five patients without chemotherapy died with a median survival of 6.2 months.

CONCLUSIONS

Colorectal neuroendocrine tumors are extremely rare showing aggressive behavior biologically, i.e fulminant early distant metastasis. Nevertheless, improved survival may be achieved by aggressive multimodality therapy.

摘要

背景/目的:结直肠神经内分泌癌是一种罕见的肿瘤,进展迅猛,预后较差。本研究旨在验证结直肠神经内分泌癌的临床病理特征。

方法

1997年6月至2004年12月在峨山医学中心,最初有10例患者基于苏木精-伊红染色(H&E)和免疫组化染色(IHC)被确诊为结直肠神经内分泌癌。本研究排除了类癌肿瘤。对13例患者的病历进行了回顾性分析。

结果

在4512例结直肠癌患者中,确诊了10例(0.2%)结直肠神经内分泌肿瘤;10例神经内分泌癌和3例伴有神经内分泌分化的腺癌。他们的中位年龄为60岁(41 - 83岁)。研究对象包括6名男性和7名女性。9个肿瘤位于直肠,2个位于乙状结肠,横结肠和盲肠各有1个。10例神经内分泌癌中有9例表达突触素,但嗜铬粒蛋白A仅在4例中表达。所有患者在诊断时均为晚期,美国癌症联合委员会(AJCC)TNM分期:IIIB期(n = 2),IIIC期(n = 3)和IV期(n = 8)。10例神经内分泌癌和3例伴有神经内分泌分化的腺癌的中位生存期分别为16.4个月和30个月。接受化疗的5例患者的中位生存期为32个月(III期)和17.5个月(IV期),而其他5例未接受化疗的患者中位生存期为6.2个月死亡。

结论

结直肠神经内分泌肿瘤极为罕见,具有生物学侵袭性,即早期迅速发生远处转移。然而,积极的多模式治疗可能提高生存率。

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Synchronous occurrence of neuroendocrine colon carcinoma and hairy cell leukemia.神经内分泌结肠癌与毛细胞白血病的同步发生。
J Gastrointest Cancer. 2011 Sep;42(3):131-6. doi: 10.1007/s12029-010-9167-7.