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[结直肠两性癌的临床病理特征]

[Clinicopathological features of colorectal amphicrine carcinoma].

作者信息

Li Z W, Sun Q, Zheng Z, Yang L L, He L, Chen D N, Zhang B, Wu H Y, Huang W B, Fan X S

机构信息

Department of Pathology, Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School, Nanjing 210008, China.

Department of Pathology, Nanjing First Hospital, Nanjing Medical University, Nanjing 210006, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2022 Aug 8;51(8):708-712. doi: 10.3760/cma.j.cn112151-20220106-00007.

Abstract

To investigate the clinicopathological, immunophenotypic and molecular features of colorectal amphicrine carcinoma (AC). Eight cases of colorectal AC were collected at the Nanjing Drum Tower Hospital and Nanjing First Hospital, Nanjing, China from 2013 to 2020. The histopathological, immunohistochemical and molecular features were analyzed. The relevant literature was reviewed. There were 6 males and 2 females, with an average age of 56 years (range 28-80 years). The tumor sites were as follows: 4 cases in sigmoid colon, 3 cases in rectum, and 1 case in transverse colon. Microscopically, there were three different patterns in the tumors, including nests with collagen hyperplasia, sheets of cells with scant stroma, and glandular or cribriform growth of goblet- or signet ring-like cells. The tumor cells generally had abundant cytoplasm with abundant mucin or eosinophilic granules. The nuclei were oval or irregular with fine chromatin and inconspicuous nucleoli. Mitotic figures were common. Neuroendocrine granules and mucin granules could be identified clearly under electron microscope. All cases showed frequent perineural and lymphovascular invasions, lymphatic metastasis, and advanced stage. Regarding immunohistochemical and specific stains, the tumor cells expressed more than two neuroendocrine markers, particularly CD56 and synaptophysin which were diffusely positive in 7 of the 8 cases. They also showed intracellular mucin in the amphicrine components which was positive for D-PAS. KRAS G12C or NRAS Q61 gene mutations were found in 2 patients. Among the six cases with complete follow-up, four of them died of the disease within three years of the diagnoses, while two were alive without known disease progression. Colorectal AC is a rare, distinct entity with both epithelial and neuroendocrine differentiation. It mainly occurs in the sigmoid colon and rectum. It typically has aggressive clinical courses, dismal prognosis and characteristic histological features and immunophenotype, which highlight the importance of recognizing this entity for clinicians and pathologists.

摘要

探讨结直肠两性癌(AC)的临床病理、免疫表型及分子特征。2013年至2020年期间,在中国南京鼓楼医院和南京第一医院收集了8例结直肠AC病例。对其组织病理学、免疫组化及分子特征进行分析,并复习相关文献。患者中男性6例,女性2例,平均年龄56岁(范围28 - 80岁)。肿瘤部位如下:乙状结肠4例,直肠3例,横结肠1例。镜下,肿瘤有三种不同模式,包括伴有胶原增生的巢状结构、细胞片状且间质稀少、杯状或印戒样细胞呈腺管状或筛状生长。肿瘤细胞通常胞质丰富,含有丰富的黏液或嗜酸性颗粒。细胞核呈椭圆形或不规则形,染色质细腻,核仁不明显。核分裂象常见。电镜下可清晰识别神经内分泌颗粒和黏液颗粒。所有病例均显示频繁的神经周围和脉管侵犯、淋巴转移及晚期病变。免疫组化及特殊染色方面,肿瘤细胞表达两种以上神经内分泌标志物,尤其是CD56和突触素,8例中有7例弥漫阳性。两性成分中还显示细胞内黏液,对D-PAS呈阳性。2例患者发现KRAS G12C或NRAS Q61基因突变。在6例有完整随访的病例中,4例在诊断后3年内死于该病,2例存活且无已知疾病进展。结直肠AC是一种罕见的独特实体,具有上皮和神经内分泌分化。主要发生在乙状结肠和直肠。其临床病程通常侵袭性强,预后不佳,具有特征性的组织学特征和免疫表型,这凸显了临床医生和病理医生认识该实体的重要性。

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