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肺上皮-肌上皮瘤:一个涉及分子组织发生的病例报告。

Epithelial-myoepithelial tumour of the lung: a case report referring to its molecular histogenesis.

机构信息

Department of Pathology, Hospital Universitario Miguel Servet Zaragoza, Spain.

出版信息

Diagn Pathol. 2011 Jul 28;6:71. doi: 10.1186/1746-1596-6-71.

Abstract

Tracheobronchial submucous glands can be considered the pulmonary equivalent of minor salivary glands and therefore they can develop most of the tumours originated in these. Nevertheless, in spite of the wide distribution of this kind of glands along the tracheobronchial tree, pulmonary salivary gland-like neoplasms are not very frequent. Among them, the most frequent are mucoepidermoid and adenoid cystic carcinomas. On the contrary, pulmonary neoplasms showing a mixture of epithelial and myoepithelial elements are extraordinary infrequent, with only 11 cases collected from literature.We present the case of a 76 year-old woman with no interesting pathological history, to whom a pulmonary nodule is detected during a study of unknown origin neutropenia. An upper right lobectomy is performed.After macro and microscopic study, the diagnosis of pulmonary epithelial-myoepithelial tumour is made. It is a low malignant potential tumour with capacity to locally recur and less frequently to metastasize. Our case has the peculiarity of not being connected neither to visceral pleura nor to bronchial tree; we have not found this characteristic in any literature reviewed case.These tumours have been named in a lot of different ways, including adenomyoepithelioma, epithelial-myoepithelial tumour, epithelial-myoepithelial carcinoma or epithelial-myoepithelial tumour of uncertain malignant potential.The p27/kip-1 protein plays a fundamental role in the development of these neoplasms. As we have verified in our case, its aberrant cytoplasmic location, besides its proved oncogenic function, would favour the proliferation of stem cells, which would explain both dual phenotype with presence of myoepithelial cells without connection with the bronchial tree, and TTF-1 immunostaining in epithelial cells.

摘要

气管支气管黏膜下腺可被视为肺部小唾液腺的等同物,因此它们可以发展为大多数起源于这些腺体的肿瘤。然而,尽管这些腺体广泛分布于气管支气管树中,肺唾液腺样肿瘤并不常见。其中,最常见的是黏液表皮样癌和腺样囊性癌。相反,显示上皮和肌上皮混合成分的肺肿瘤非常罕见,从文献中仅收集到 11 例。我们报告了一例 76 岁女性病例,无有趣的病史,在一项不明原因中性粒细胞减少症的研究中发现了肺结节。行右上肺叶切除术。经过宏观和微观研究,诊断为肺上皮-肌上皮肿瘤。这是一种低度恶性潜能的肿瘤,具有局部复发的能力,较少发生转移。我们的病例的特点是既不与内脏胸膜也不与支气管树相连;在我们回顾的文献中没有发现这种特征。这些肿瘤有很多不同的命名方式,包括腺肌上皮瘤、上皮-肌上皮肿瘤、上皮-肌上皮癌或恶性潜能不确定的上皮-肌上皮肿瘤。p27/kip-1 蛋白在这些肿瘤的发生中起着重要作用。正如我们在本病例中所验证的,其异常的细胞质定位,除了已证明的致癌功能外,还会促进干细胞的增殖,这可以解释同时存在肌上皮细胞而与支气管树无联系的双重表型,以及上皮细胞中 TTF-1 的免疫染色。

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