肾脏球旁细胞瘤一例并文献复习

Juxtaglomerular Cell Tumor: A Case Study and Literature Review

摘要: 目的：探讨肾脏球旁细胞瘤(juxtaglomerular cell tumor, JGCT)临床病理学特征、免疫组化、病理学诊断、鉴别诊断和治疗方法。方法：回顾性分析一例JGCT病例临床资料、组织学特征、免疫组化染色及特殊染色，同时复习相关文献。结果：患者，男，46岁，临床表现主要为高血压及低血钾。镜下肿瘤与周围肾组织界限较清，肿瘤细胞呈实性巢片或条索状排列，细胞大小一致，呈圆形或卵圆形，无异型性，巢索间血管裂隙丰富，血窦样，部分区域可见透明变性的厚壁血管。免疫组化示肾素、CD34、SMA弥漫阳性；PAS染色阳性。此肿瘤可能与发生于肾脏的其它肿瘤混淆，需要与多种肿瘤相鉴别。患者肾脏切除后，血压恢复正常，全身状况良好。结论：肾脏球旁细胞瘤是一种罕见的良性肾脏肿瘤，易误诊、漏诊，病理医师需全面掌握其组织病理学形态、免疫组化特点及鉴别诊断要点，方能提高诊断准确性。

Abstract: Objective: This study aims to investigate the clinicopathological features, immunohistochemical characteristics, pathological diagnosis, differential diagnosis, and therapeutic approaches for juxtaglomerular cell tumor (JGCT). Methods: A retrospective analysis was performed on the clinical data, histological features, immunohistochemical staining, and special staining of a JGCT case, supplemented by a review of pertinent literature. Results: The patient, a 46-year-old male, exhibited hypertension and hypokalemia as primary clinical symptoms. Microscopically, the tumor was well-demarcated from the adjacent renal tissue. Tumor cells were organized in solid nests or cords, displaying uniform size and round to oval shapes without atypical features. Numerous vascular clefts and sinusoid-like spaces were noted between the nests, along with regions of hyalinized thick-walled blood vessels. Immunohistochemical analysis revealed diffuse positivity for Renin, CD34, and SMA, with PAS staining also positive. Differential diagnosis is necessary to distinguish this tumor from other renal neoplasms. Following nephrectomy, the patient’s blood pressure normalized, and his overall condition improved. Conclusion: The JGCT represents a rare benign neoplasm of the kidney, frequently subject to diagnostic challenges, including misdiagnosis and oversight. It is imperative for pathologists to possess a thorough understanding of its histopathological characteristics, immunohistochemical profile, and critical aspects of differential diagnosis to enhance diagnostic precision.