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支气管黏液表皮样癌:综述

Mucoepidermoid carcinoma of the bronchus: a review.

作者信息

Liu Xiuli, Adams Amy L

机构信息

Department of Pathology, University of Alabama at Birmingham, USA.

出版信息

Arch Pathol Lab Med. 2007 Sep;131(9):1400-4. doi: 10.5858/2007-131-1400-MCOTBA.

Abstract

Although mucoepidermoid carcinoma of the salivary gland is relatively common, mucoepidermoid carcinoma arising from the mucous glands of the bronchus is rare. Bronchial mucoepidermoid carcinoma usually presents as an intraluminal mass producing luminal occlusion. Symptoms are airway obstruction and recurrent pneumonia. Macroscopically, mucoepidermoid carcinoma appears as an exophytic intrabronchial mass with intact or ulcerated bronchial mucosa. Microscopically, the tumors are located in the submucosa of the large bronchi. The tumors are usually well differentiated and contain a combination of mucus-secreting, squamous, and intermediate cells. The increased frequency of this tumor in the pediatric population suggests a genetic abnormality. Recent genetic studies have demonstrated reciprocal chromosomal translocations including t(1;11)(p22;q13), t(11;19)(q14-21;p12), and t(11; 19)(q21;p13). Chromosome 11 in the first translocation appears to have been altered resulting in up-regulation of the cyclin D1 gene and overexpression of cyclin D1. The t(11;19)(q21;p13) encodes a novel fusion product capable of disrupting the Notch signaling pathway.

摘要

尽管涎腺黏液表皮样癌相对常见,但起源于支气管黏液腺的黏液表皮样癌却很罕见。支气管黏液表皮样癌通常表现为腔内肿块导致管腔阻塞。症状为气道阻塞和反复肺炎。大体上,黏液表皮样癌表现为支气管内突出性肿块,支气管黏膜完整或溃疡。显微镜下,肿瘤位于大支气管黏膜下层。肿瘤通常分化良好,包含黏液分泌细胞、鳞状细胞和中间细胞的组合。该肿瘤在儿童人群中发病率增加提示存在基因异常。最近的基因研究已证实存在相互的染色体易位,包括t(1;11)(p22;q13)、t(11;19)(q14 - 21;p12)和t(11;19)(q21;p13)。第一次易位中的11号染色体似乎发生了改变,导致细胞周期蛋白D1基因上调和细胞周期蛋白D1过度表达。t(11;19)(q21;p13)编码一种能够破坏Notch信号通路的新型融合产物。

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