肾球旁细胞瘤2例临床病理探讨

目的分析肾球旁细胞瘤的临床病理特征,探讨其生物学行为及预后因素。方法采用光镜和免疫组化方法,结合临床资料对比研究2例肾球旁细胞瘤的临床病理特点。结果 2例肾球旁细胞瘤患者均为男性,年龄分别为46岁和9岁,有高血压症状;均行肾部分切除术,术后随访1～6个月,无复发或转移。肿瘤位于肾皮质,直径2 cm,界清。例1具有典型的良性肾球旁细胞瘤特征,即瘤组织实性、片状分布,细胞呈圆形,大小较一致,胞质淡染或弱嗜酸性,胞核呈圆形或椭圆形,染色质细腻,缺乏核分裂;间质毛细血管和血窦丰富,透明变性的厚壁血管呈簇状分布。例2则由椭圆形、多角形、梭形及带状细胞构成,核异型性明显,核分裂8～10个/10HPF,可见病理性核分裂;部分区域呈席纹状或旋涡状生长。免疫组化示肿瘤细胞vimentin和CD34弥漫强(+),SMA不同程度(+);例1和例2的Ki-67指数分别为<1%和20%。结论肾球旁细胞瘤是一种良性肿瘤,少部分病例为恶性潜能未定。提示恶性潜能的临床病理指标包括血管或包膜浸润、肿瘤体积大、高核分裂象及年长或年幼患者。明显核异型对预后的影响尚不确定。

Clinicopathologic features of juxtaglomerular cell tumor: a study of two cases

Objective To analyze the clinicopathologic features of juxtaglomerular cell tumor of the kidney(JGCT) and to discuss its biological behaviour and prognostic factors.Methods The clinical,pathological and immunohistochemical features were evaluated in 2 cases of JGCT.Results The JGCT came from 2 male patients(age at diagnosis,46 vs.9 years) with symptoms of systemic hypertension.Both of them underwent partial nephrectomy and followed up for 1 to 6 months.There were no recurrence or metastases.Tumors were both located in the renal cortex with the diameter of 2 cm and well-circumscribed.Case 1 showed the typical benign histological features of JGCT;the tumor consisted of solid sheets of closely packed uniform round cells with oval to round nuclei,pale to lightly eosinophilic cytoplasm,inconspicuous nucleoli and indistinct cell border,and mitotic figure was not identified;interstitium riched in thin-walled capillaries,and hyalinized thick-walled blood vessels were grouped in small clusters.Whereas the tumor in case 2 consisted of oval to polygonal to spindled/elongated cells with nuclei in variable size;unusual features included prominent nuclear atypia,high mitotic count(8-10/10 high-power fields),atypical mitotic figures and storiform/whorled growth pattern.Immunohistochemically,both JGCTs were strongly and diffusely positive for CD34 and vimentin,partial or focal staining for SMA,but showed various Ki-67 index(case 1 vs.case 2,<1% vs.20%).Conclusions JGCT should be considered as a tumor with uncertain malignant potential.Clinicopathogical signs of potential malignancy in JGCT include histological vascular invasion,a large tumor size,common mitosis activity,and relatively advanced/younger age of patients.The relationship between nuclear atypia and prognosis has not been identified yet.