5例嗜酸性实性和囊性肾细胞癌的临床病理特征OA
Clinicopathological features of 5 cases of eosinophilic solid and cystic renal cell carcinoma
目的:嗜酸性实性和囊性肾细胞癌(eosinophilic solid and cystic renal cell carcinoma,ESC RCC)是第5版世界卫生组织(World Health Organization,WHO)肾肿瘤分类中新增的一种罕见类型,镜下呈实性与囊性混合结构,细胞质丰富且呈嗜酸性.鉴于该肿瘤临床少见、认知尚有限,本研究探讨ESC RCC的临床表现、病理形态学特征、免疫组织化学(以下简称"免疫组化")特点及鉴别诊断要点,为临床精准诊断、个性化治疗方案选择及预后评估提供可靠参考依据.方法:回顾性收集2022年6月至2025年5月安徽医科大学第一附属医院泌尿外科经手术切除并经病理确诊的5例ESC RCC患者的完整临床病理资料及术后随访数据.结合显微镜下的组织学结构、细胞形态特征及免疫组化分析其临床病理学特征,并系统地复习国内外相关文献进行综合分析.结果:5例患者均为女性,中位年龄为61岁.4例因腰部隐痛就诊,疼痛程度较轻且呈间歇性发作,无明显放射痛,无其他伴随症状;1例因体检行腹部影像学检查偶然发现肾占位,无任何临床症状.影像学检查结果显示,5例肿瘤均为单侧单发,均位于左肾皮质区,与肾髓质界限清晰,未侵及肾盂及肾周脂肪组织.肿瘤最大径为4.8~6.7 cm,平均为5.8 cm.平扫呈混杂或稍高密度影,增强扫描后呈轻中度不均匀强化.大体标本显示,肿瘤呈灰黄色或棕褐色,边界清晰,无明显包膜.镜下观察显示,肿瘤组织以实性生长为主,局部区域可见大小不等的微囊结构;肿瘤细胞呈多边形或圆形,排列成巢状、片状,细胞界限清晰,细胞质丰富且呈强嗜酸性,部分细胞质内可见细小颗粒状嗜碱性彩斑,为该肿瘤的特征性形态表现;细胞核呈圆形或椭圆形,核仁不明显,核分裂象罕见[≤1个/10高倍视野(high power field)],其中有1例(例3)可见脉管内癌栓.免疫组化结果显示,5例患者肿瘤细胞均不同程度表达细胞角蛋白(cytokeratin,CK)20(细胞质阳性)、配对盒基因(paired box gene,PAX)8(细胞核阳性)、α-甲基酰基辅酶 A 消旋酶(alpha-methylacyl-CoA racemase,AMACR)(细胞质阳性)、琥珀酸脱氢酶B亚基(succinate dehydrogenase subunit B,SDHB)(细胞质阳性)及波形蛋白(vimentin)(细胞质阳性),CK7、人黑色素瘤黑色素(human melanoma black,HMB)45均为阴性表达;1例患者局灶性表达分化簇117(cluster of differentiation 117,CD117)及碳酸酐酶Ⅸ(carbonic anhydrase Ⅸ,CA-Ⅸ)细胞膜阳性.2例患者行根治性肾切除术,3例患者行肾部分切除术,5例患者术后均未发生出血、感染、尿瘘等严重并发症,均顺利出院.术后随访至2025年12月,随访时间6~42个月,其中1例失访,余4例均存活,均未发现肿瘤复发或转移征象.结论:ESC RCC好发于中年女性,具有典型的"实性+囊性"组织形态及"CK20+/CK7-"的免疫表型特征,免疫组化检测可有效与其他嗜酸性肾肿瘤鉴别,手术切除后预后良好.
Objective:Eosinophilic solid and cystic renal cell carcinoma(ESC RCC)is a rare tumor entity newly included in the 5th edition of the World Health Organization classification of renal tumors.Histologically,it is characterized by a mixed solid and cystic architecture with abundant eosinophilic cytoplasm.Given its rarity and limited current understanding,this study aims to investigate the clinical presentation,histopathological features,immunohistochemical characteristics,and differential diagnostic points of ESC RCC,thereby providing a reliable reference for accurate diagnosis,individualized treatment,and prognostic evaluation. Methods:Five patients with ESC RCC who underwent surgical resection and were pathologically confirmed in the Department of Urology at the First Affiliated Hospital of Anhui Medical University from June 2022 to May 2025 were retrospectively included.Complete clinicopathological data and postoperative follow-up information were collected.The clinicopathological features were analyzed based on histological architecture,cytological morphology,and immunohistochemical findings,and relevant domestic and international literature was systematically reviewed. Results:All 5 patients were female,with a median age of 61 years.Four patients presented with mild,intermittent dull flank pain without radiation or accompanying symptoms.One case was incidentally detected during routine physical examination by abdominal imaging and was asymptomatic.Imaging studies revealed that all tumors were unilateral and solitary,located in the cortical region of the left kidney,with clear boundaries from the renal medulla and no invasion of the renal pelvis or perirenal fat.The maximum tumor diameter ranged from 4.8 to 6.7 cm,with a mean maximum diameter of 5.8 cm.On non-contrast imaging,lesions showed heterogeneous or slightly hyperdense signals;contrast-enhanced scans demonstrated mild to moderate heterogeneous enhancement.Grossly,tumors appeared gray-yellow or brownish,well circumscribed,without an obvious capsule.Microscopically,tumors predominantly exhibited solid growth with focal microcystic structures of varying sizes.Tumor cells were polygonal or round,arranged in nests or sheets,with well-defined cell borders and abundant intensely eosinophilic cytoplasm.Fine granular basophilic stippling was observed in some cytoplasm,representing a characteristic morphological feature of this tumor.Nuclei were round or oval with inconspicuous nucleoli,and mitotic figures were rare[≤1/10 high-power fields(HPF)].One case showed vascular tumor thrombus.Immunohistochemically,tumor cells in all 5 cases showed varying degrees of positivity for cytokeratin(CK)20(cytoplasmic),paired box gene 8(PAX8)(nuclear),alpha-methylacyl-CoA racemase(AMACR)(cytoplasmic),succinate dehydrogenase subunit B(SDHB)(cytoplasmic),and vimentin(cytoplasmic),while CK7 and human melanoma black 45(HMB45)were negative.One case showed focal positivity for CD117(membranous)and carbonic anhydrase IX(CA-IX)(membranous).Of the 5 patients,2 underwent radical nephrectomy and 3 underwent partial nephrectomy.No serious postoperative complications such as bleeding,infection,or urinary fistula occurred,and all patients were discharged uneventfully.Follow-up was conducted until December 2025,with a duration ranging from 6 to 42 months.One patient was lost to follow-up,while the remaining 4 patients survived without evidence of recurrence or metastasis. Conclusion:ESC RCC predominantly occurs in middle-aged women and is characterized by a distinctive"solid and microcystic"histological pattern and a"CK20+/CK7-"immunophenotype.Immunohistochemistry is useful for distinguishing it from other eosinophilic renal tumors.Surgical resection is associated with a favorable prognosis.
柯晶;刘海艳
安徽医科大学第一附属医院病理科,合肥 230022安徽医科大学第一附属医院病理科,合肥 230022
医药卫生
肾细胞癌嗜酸性实性和囊性肾细胞癌临床病理特征免疫表型预后
renal cell carcinomaeosinophilic solid and cystic renal cell carcinomaclinicopathological featuresimmunophenotypeprognosis
《临床与病理杂志》 2026 (2)
287-293,7
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