Sinonasal Renal Cell-Like Adenocarcinoma: A Report of a Rare Entity With Emphasis on Its Association With Von Hippel Lindau Syndrome

鼻窦肾细胞样腺癌:一例罕见病例报告及其与冯·希佩尔-林道综合征的关联

阅读:1

Abstract

Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare malignant sinonasal tumor with relatively indolent clinical course. Clinically, it could be asymptomatic or show non-specific symptoms such as epistaxis, nasal obstruction, or hyposmia. Diagnosis of the lesion is challenging, especially in small biopsies, and requires clinical, radiological, histopathological, and ancillary tests to characterize the lesion accurately. We herein report a case of a 41-year-old female with a nasal mass noted two years ago, which presented initially as frequent epistaxis from the right side. Histopathological examination revealed proliferation of clear cells associated with hemorrhagic background forming follicular and glandular structure and dense eosinophilic secretion. Tumor cells were diffusely positive for CK7, EMA, and inhibin, while they were negative for CK20, P63, CK 5/6, CD10, renal cell carcinoma (RCC), TTF1, PAX8, CEA, and GATA3. The proliferation index (KI67) was less than 5%. The diagnosis was consistent with SNRCLA. The patient has no recurrence and no symptoms after one year. Thus, our study reports a rare case of SNRCLA with a discussion of the histological features and its association with von Hippel Lindau syndrome.

特别声明

1、本页面内容包含部分的内容是基于公开信息的合理引用;引用内容仅为补充信息,不代表本站立场。

2、若认为本页面引用内容涉及侵权,请及时与本站联系,我们将第一时间处理。

3、其他媒体/个人如需使用本页面原创内容,需注明“来源:[生知库]”并获得授权;使用引用内容的,需自行联系原作者获得许可。

4、投稿及合作请联系:info@biocloudy.com。