嗜酸性实质囊性肾细胞癌（ESC-RCC）是一种被低估的新型散发性肾肿瘤，被列为2022 WHO肾肿瘤分类中一种罕见的肾细胞癌。由于其特征不够清楚，易被误诊。我们报告了一例ESC-RCC的病例，患者是一名53岁的女性，临床检查发现右肾有一肿块，但无不适症状。我们泌尿科进行计算机断层扫描，显示右肾周围有一圆形软组织密度阴影。显微镜检查显示，肿瘤显示出嗜酸性细胞的实质囊性组成，具有独特的免疫组化标记（CK20阳性/CK7阴性）和TSC2基因的无意义突变。在肾肿瘤切除术后的十个月中，患者身体状况良好，没有复发或转移。我们在这里描述的ESC-RCC的明显形态、免疫表型和分子特征，基于我们的病例和相关文献，突出了这种新型肾肿瘤的病理学和鉴别诊断的关键点。因此，我们的研究将加深我们对这种新型肾肿瘤的理解，并有助于减少误诊。© 2023. 作者。

Eosinophilic solid and cystic renal cell carcinoma (ESC-RCC) is an under-recognized, emerging new entity of sporadic renal neoplasms, which is listed as a rare type of renal cell carcinoma in the 2022 WHO renal tumor classification. It is easily misdiagnosed because its characteristics are insufficiently understood.We report one case of ESC-RCC, a 53-year-old female patient with a right kidney mass found during clinical examination. The patient experienced no discomforting symptoms. Computer-tomography imaging at our urinary department showed a round soft tissue density shadow around the right kidney. Microscopic examination revealed a tumor displaying a solid-cystic composition of eosinophilic cells with unique features, revealed by characteristic immunohistochemical markers (CK20-positive/CK7-negative), and a nonsense mutation in TSC2. Ten months after the renal tumor resection, the patient presented in good condition with no recurrence or metastasis.The distinct morphological, immunophenotypic, and molecular characteristics of ESC-RCC we describe here, based on our case and the relevant literature, highlight the key points of the pathological and differential diagnosis of this novel renal neoplasm. Our findings will therefore deepen our understanding of this novel renal neoplasm and help reduce misdiagnosis.© 2023. The Author(s).