特发性肾脏替代性脂肪瘤(附1例报告并文献复习)

目的探讨特发性肾脏替代性脂肪瘤(RRL)的临床特点、诊断和治疗方法。方法回顾性分析1例肾脏替代性脂肪瘤患者的临床资料。男性,48岁,体检发现左肾占位入院,超声提示左肾实质破坏,肾盂内强回声团伴声影,左肾被高回声软组织影包裹。CT提示左肾占位,可见明显负值,无明显强化,肾盂内可见高密度影,肾实质不均匀破坏。结果患者行手术治疗,术中探查见左肾被巨大脂肪组织包裹,行肾脏切除术。病理所见患肾皮质高度萎缩,肾脏体积明显增大,肾脏被过度增生的脂肪纤维组织所替代,光镜下可见到大量异常肥大的脂肪细胞。患者术后1周痊愈出院,随访18个月未见肿物复发转移。结论 RRL临床极为罕见,目前国内外文献仅见少数个案报道。RRL需要与慢性感染性肾脏疾病、脂肪瘤、脂肪肉瘤、血管平滑肌脂肪瘤和畸胎瘤等含有脂肪组织的肾脏肿瘤、黄色肉芽肿性肾盂肾炎、肥胖及Cushing综合征等疾病相鉴别。治疗以手术为主,通常需要行肾脏切除术。

Idiopathic renal replacement lipomatosis: a case report and review of literature

Objective To study the clinical features,diagnosis and treatment of idiopathic renal replacement lipomatosis（RRL）.Methods The clinical data of one patient with RRL was analyzed retrospectively.A 48-year old man was admitted to the hospital for renal lesion in routine physical examination.The ultrasonography showed hyperechoic lesion around left kidney.CT scanning revealed an enlarged kidney with irregular parenchyma,surrounded by abundant low-density fat.Results The patient underwent nephrectomy.The kidney was almost replaced by fatty tissues.Pathological examination showed hyperplastic fatty tissues at the renal sinus and hilus,and atrophic renal cortex.The patient was well with no evidence of recurrent tumor after operation during 18 months follow-up.Conclusion Renal replacement lipomatosis is a rare benign renal disease.Although rare,it should be the differential diagnosis of the renal tumors,especially those fatty tumors.