机构：[1]Department of Pathology, The First People's Hospital of Yunnan Province, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, China.医技片病理科云南省第一人民医院[2]Department of Radiology, The First People's Hospital of Yunnan Province, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, China.医技片放射科云南省第一人民医院[3]Department of Gastroenterology, The First People's Hospital of Yunnan Province, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, China.内科片消化内科云南省第一人民医院[4]Department of Nuclear Medicine, Peking University Cancer Hospital Yunnan, Yunnan Cancer Hospital, The Third Affiliated Hospital of Kunming Medical University, Kunming, China.
Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare, low-grade malignant neoplasm originating in the nasal cavity and paranasal sinuses. This report provides a detailed account of the clinical and pathological characteristics of a single case of SNRCLA, highlights its differential diagnosis, and includes a review of relevant literature.A 13-year-old male presented with a mass in the right nasal cavity. Histopathological analysis demonstrated tumor morphology analogous to clear cell renal cell carcinoma, characterized by abundant clear cytoplasm, acinar formations, and a prominent capillary-rich stroma. Immunohistochemical analysis revealed positive staining for cytokeratin pan, cytokeratin 7, vimentin, carbonic anhydrase IX, and SRY-box 10, with partial positivity for S-100. Markers such as paired box 8, atriopeptidase, renal cell carcinoma marker, melanosome, thyroglobulin, and thyroid transcription factor-1were negative. Fluorescence in situ hybridization analysis identified the presence of an EWSR1 gene rearrangement. The patient underwent surgical resection followed by adjuvant radiotherapy. Follow-up evaluations conducted as of November 2024 revealed no evidence of tumor recurrence, and the patient remained in good general health.SNRCLA is a rare neoplasm characterized by its low incidence rate and the necessity for exclusion-based diagnosis, requiring differentiation from other clear cell tumors. Immunohistochemistry plays a key role in establishing the diagnosis and distinguishing SNRCLA from histologically-similar entities. This case represents the first documentation of EWSR1 gene rearrangement in SNRCLA, thereby contributing novel insights into its molecular profile.
基金：
The author(s) disclosed receipt of the following financial support
for the research, authorship, and/or publication of this article:
This study was funded by the Yunnan Digestive Endoscopy
Clinical Medical Center Foundation for Health Commission of
Yunnan Province (Grant Number: 2022LCZXKF-XH10). The
funding body had no role in the design of the study and collection,
analysis, and interpretation of data and in writing the
manuscript.
第一作者机构：[1]Department of Pathology, The First People's Hospital of Yunnan Province, The Affiliated Hospital of Kunming University of Science and Technology, Kunming, China.
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推荐引用方式(GB/T 7714)：
Tang Ming,Yao Hong,Yang Chao,et al.Case Report of a Primary Sinonasal Renal Cell-Like Adenocarcinoma With EWSR1 Gene Rearrangement in a 13-Year-Old Male[J].Ear, Nose, & Throat Journal.2025,1455613251323776.doi:10.1177/01455613251323776.
APA：
Tang Ming,Yao Hong,Yang Chao,Liu Peng-Jie&Yang Xuan-Tao.(2025).Case Report of a Primary Sinonasal Renal Cell-Like Adenocarcinoma With EWSR1 Gene Rearrangement in a 13-Year-Old Male.Ear, Nose, & Throat Journal,,
MLA：
Tang Ming,et al."Case Report of a Primary Sinonasal Renal Cell-Like Adenocarcinoma With EWSR1 Gene Rearrangement in a 13-Year-Old Male".Ear, Nose, & Throat Journal .(2025):1455613251323776
