肾球旁细胞瘤4例报道并文献复习

Juxtaglomerular cell tumor:a report of 4 cases and literature review

目的探讨肾球旁细胞瘤的症状、影像学表现、诊断、病理、治疗及预后。方法通过回顾分析4例肾球旁细胞瘤(JGCT)的临床资料,结合相关文献进行讨论。结果 4例患者平均年龄25.5岁,术前均为高血压。3例入院前为低血钾、高肾素、高醛固酮血症。CT呈低密度影,增强扫描可见强化,强化程度低于肾实质。MRI提示稍长T1、T2信号,边界欠清,增强后动脉期及静脉期持续不均匀强化,延迟期减低。分别行经腹腹腔镜下肾肿瘤剜除术、肾癌根治术和机器人辅助腹腔镜下肾肿瘤剜除术。肿瘤组织呈片、巢状排列,漩涡状生长。免疫组化:肿瘤细胞表达CD34(+++),SMA(散在+),HMB45(±),Syn(弱+),ki67(约4%+)。术后患者中位随访时间9个月,均未见高血压、低血钾及肿瘤复发。结论 JGCT临床罕见,术前多有高血压及低血钾症状,CT和磁共振对诊断有帮助,但确诊需组织病理结合免疫组化,可予肿瘤剜除或肾脏切除,预后良好,但极少数存在恶性潜质,需要长期随访。

Objective To investigate the symptoms,imaging,diagnosis,pathological features,treatment and prognosis of juxtaglomerular cell tumor（JGCT）.Methods Clinical data of 4 cases of JGCT were retrospectively analyzed,and relevant literature was reviewed.Results Of the 4 cases,1 was male and 3 were female,aged 15 to 37 years（mean25.5）.All cases had hypertension and 3 cases had hypokalemia,hyperaldosteronism and high renin.CT showed hypodense shadow and enhanced scan showed significant enhancement less than the renal parenchyma.MRI showed slightly longer T1 and T2 signals with unclear boundary which were heterogeneous.Enhanced scan showed continuous and heterogeneous enhancement in arterial,venous phases and abatement in delayed phase.Partial nephrectomy of the kidney,endoscopic robotic-assisted enucleation and radical nephrectomy were performed.The Tumor tissues were arranged in nesting and lamellate pattern and showed swirling growth.Immunohistochemical results showed tumor cells were positive for CD34,vimentin,SMA,CD40,HMB45 and ki67.During the median follow-up of 9 months,no hypokalemia,hypertension or recurrence occurred.Conclusions JGCT is a rare disease with hypokalemia,hypertension,hyperaldosteronism and high renin.CT and MRI are important for the diagnosis,but a confirmed diagnosis requires pathological and immunohistochemical tests.Surgery is the major treatment method.JGCT has great prognosis but needs long-term follow-up because of its malignant potential.