右肾肿块为首发表现的多系统软斑症诊治分析

A case report of multi-system malacoplakia and analysis of diagnosis and treatment

目的:探讨软斑症的发病机制、临床表现、病理特征、诊断方法及治疗策略。方法:通过报道1例肾、肝、结肠多系统软斑症的诊断与治疗经过,并结合现有文献对本病进行探讨。患者女性,65岁,因"右侧腰腹部疼痛1个月"就诊,术前影像学检查提示:右肾占位性病变,考虑肾癌并感染可能性大,侵犯结肠肝曲,累及肝右叶。结果:软斑症患者经过超声引导下穿刺活检,腹腔镜下右肾根治性切除+肝部分切除+结肠肝曲切除术切除病变组织,术后病理提示:右侧肾、肝及结肠软斑病。配合抗感染治疗,术后恢复良好,顺利出院。术后1个月随访,患者无腰痛及发热表现,皮疹已消退。复查各项指标正常。全腹增强CT提示:右肾术后缺如,右肾上腺部分切除显示不清;肝右叶下段部分缺如;右侧后腹膜腔少量包裹性积液,腹膜增厚。结论:软斑症非常罕见,多系统软斑症的发病率更低,需要结合临床表现、影像学、分子病理学等进行诊断,该疾病与宿主免疫缺陷有关。对于多系统软斑症,手术仍是积极有效的治疗措施,术后需要长期应用抗生素治疗,能改善症状,但易于复发。

Objective To discussthe pathogenesis,clinical manifestations,pathological features,diagnosis methods and treatment strategies of malacoplakia.Methods Through the report of a case of diagnosis and treatment of multi-system malacoplakia of the kidney,liver and colon,the disease was discussed in combination with the existing literature.A 65-year-old female patient presented to the doctor with"right side waist and abdomen pain for I month".Preoperative imaging examination revealed:right kidney space-occupying lesions,considering the possibility of renal cancer and infection,invasion of liver ffexure of the colon,and involvement ofthe right lobe of the liver.ResultsThe patient with malacoplakia underwent ultrasound-guided puncture biopsy,laparoscopic radical resection of the right kidney and partial liver resection and colon hepatectomy to remove the diseased tissue.Postoperative pathology showed:right kidney,liver and colonic soft spot disease.Cooperate anti-infctive treatment,the patient got recovery and discharged smoothly.one month after operation,the patient had no back pain and fever,and the rash had subsided.All indicators rechecked were normal.The enhanced CT of the whole abdomen showed that the right kidney was absent afer the operation,and the right adrenal gland was unclear after partial resection;the lower part of the right liver lobe was absent;a smallamount of encapsulated fuid in the right posterior peritoneal cavity and thickened peritoneum.Conclusion Malacoplakia is very rare,and the incidence of multi-system malacoplakia is lower.It needs to be diagnosed in combination with clinical manifestations,imaging,and molecular pathology.The disease is related to host immune deficiency.For multiple system malacoplakia,surgery is stll an ffective treatment.Longterm antibiotic treatment is required after surgery which can improve symptoms,but is prone to relapse.