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原发性肺涎腺导管癌:临床病理特征、诊断及实际挑战

Primary salivary duct carcinoma of the lung: clinicopathological features, diagnosis and practical challenges.

作者信息

Li Shaoling, Hou Likun, Huang Yan, Wu Wei, Wu Chunyan, Zhang Liping

机构信息

Department of Pathology, Shanghai Pulmonary Hospital, School of Medicine,Tongji University, Shanghai 200433, China.

Department of Pathology, Shanghai Pulmonary Hospital, School of Medicine,Tongji University, Shanghai 200433, China

出版信息

J Clin Pathol. 2024 Apr 18;77(5):324-329. doi: 10.1136/jcp-2022-208671.

Abstract

AIMS

To investigate the clinicopathological features, molecular characteristics and diagnostic criteria of primary salivary duct carcinoma of the lung (LSDC).

METHODS

We analysed the clinicopathological and molecular features of five cases of LSDC retrieved from the archives of Shanghai Pulmonary Hospital from 2020 to 2022, and reviewed the relevant literature.

RESULTS

All patients were men, with an average age of 66 years (age range: 49-79 years), and all lesions were central masses with a mean maximum diameter of 42.6 mm (range: 16-70 mm). Morphologically, LSDC comprised of intraductal and invasive components. Both the intraductal and invasive components of LSDC can exhibit papillary, micropapillary, cribriform, tubule structures and solid proliferation. The intraductal component can exhibit Roman bridge structures, which were usually accompanied by central comedo-like necrosis. Immunohistochemically, LSDCs consistently expressed cytokeratin (CK)7 (5 of 5) and showed variable positivity of androgen receptor (AR) (5 of 5) focally or diffusely; additionally, the tumour cells expressed human epidermal growth factor receptor 2 (HER2) (3+, n=3; 2+, n=2), GATA-binding protein 3 (3 of 5), and gross cystic disease fluid protein-15 (1 of 5), and all of which were negative for thyroid transcription factor-1, napsin A, p40, CK5/6 and p63. The residual basal/myoepithelial cells surrounding the in situ carcinoma expressed p40, CK5/6 and p63. TP53 mutation and HER2 gene amplification (3 of 5) were the most frequent genetic alterations in LSDC. All patients who underwent surgical lobectomies were alive without recurrence or metastasis.

CONCLUSIONS

LSDC is a highly rare malignant tumour. The distinctive architecture of in situ carcinoma and tumour cells expressing AR can provide diagnostic indications for LSDC.

摘要

目的

探讨肺原发性涎腺导管癌(LSDC)的临床病理特征、分子特征及诊断标准。

方法

我们分析了2020年至2022年从上海肺科医院档案中检索出的5例LSDC的临床病理和分子特征,并复习了相关文献。

结果

所有患者均为男性,平均年龄66岁(年龄范围:49 - 79岁),所有病变均为中央型肿块,平均最大直径42.6 mm(范围:16 - 70 mm)。形态学上,LSDC由导管内和浸润性成分组成。LSDC的导管内和浸润性成分均可表现为乳头状、微乳头状、筛状、小管状结构及实性增生。导管内成分可表现为罗马桥结构,通常伴有中央粉刺样坏死。免疫组化方面,LSDC始终表达细胞角蛋白(CK)7(5/5),雄激素受体(AR)呈局灶或弥漫性不同程度阳性(5/5);此外,肿瘤细胞表达人表皮生长因子受体2(HER2)(3 +,n = 3;2 +,n = 2)、GATA结合蛋白3(5/5中的3例)和大囊性病液蛋白-15(5/5中的1例),所有病例甲状腺转录因子-1、 napsin A、p40、CK5/6和p63均为阴性。原位癌周围残留的基底/肌上皮细胞表达p40、CK5/6和p63。TP53突变和HER2基因扩增(5/5中的3例)是LSDC最常见的基因改变。所有接受手术肺叶切除术的患者均存活,无复发或转移。

结论

LSDC是一种极为罕见的恶性肿瘤。原位癌独特的结构及表达AR的肿瘤细胞可为LSDC提供诊断依据。

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