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目的 探讨肾脏球旁细胞瘤的临床病理学特征、免疫组化表型，分析诊断要点。方法 回顾性分析3例肾脏球旁细胞瘤患者的临床资料、镜下形态和免疫组化，结合相关文献进行复习和探讨。结果 3例患者中女性2例，男性1例，年龄23—32岁，中位年龄为30岁，均表现为高血压和低血钾症状，2例伴有高胆固醇。所有患者均接受了肾肿瘤切除术，病理显示瘤细胞椭圆形或短梭形，间质内血窦丰富，可见肥大细胞、淋巴细胞浸润。免疫组化示瘤细胞表达CD34，不同程度表达波形蛋白（vimentin）、平滑肌肌动蛋白（smooth muscle actin,SMA）等。术后患者实验室检查指标均恢复正常。结论 肾脏球旁细胞瘤是一种主要发生于青年人的罕见良性肿瘤，以高血压及低血钾为主要临床表现，确诊需结合病理学形态及免疫表型;需与血管球瘤、血管外皮瘤等进行鉴别诊断；治疗以手术切除为主，预后较好。
Abstract：Objective To explore the clinical and pathological features, immunohistochemical phenotypes, and diagnostic key points of juxtaglomerular cell tumors(JGCTs). Methods We retrospectively analyzed the clinical data, microscopic morphology, and immunohistochemical findings of 3 patients with JGCTs, and reviewed relevant literature. Results Among the 3 patients, 2 were female and 1 was male, with median age of 30 years(range: 23—32 years). All patients presented with hypertension and hypokalemia, and 2 patients had elevated total cholesterol. All patients underwent nephrectomy for tumor removal. Pathological examination revealed that the tumor cells were oval or short spindle-shaped, with abundant sinusoids in the stroma, and infiltration by mast cells and lymphocytes. Immunohistochemistry showed that the tumor cells expressed CD34 and to varying degrees, vimentin and smooth muscle actin(SMA). Postoperatively, all laboratory test results returned to normal levels. Conclusion Juxtaglomerular cell tumor is a rare benign neoplasm predominantly affecting young adults,characterized primarily by hypertension and hypokalemia. Diagnosis requires integration of histopathological features and immunophenotypic analysis; differential diagnosis should consider conditions such as glomus tumor and hemangiopericytoma. Treatment mainly involves surgical resection, with generally favorable prognosis.
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基本信息:
DOI：10.20256/j.cnki.zgdxbl.20250516.002
中图分类号:R737.11
引用信息:
[1]王安哲,王雨纯,刘蔚.肾球旁细胞瘤3例临床病理分析[J].中国典型病例大全,2025,19(02):9-14.DOI:10.20256/j.cnki.zgdxbl.20250516.002.
基金信息: