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常染色体显性遗传性多囊肾病合并肾细胞癌:一例报告

Renal cell carcinoma in autosomal dominant polycystic kidney disease: A case report.

作者信息

Rooseno Gullyawan, Yatindra Ida Bagus Gde Tirta Yoga, Djatisoesanto Wahjoe, Djojodimedjo Tarmono

机构信息

Department of Urology, Faculty of Medicine, Airlangga University, Surabaya, East Java, Indonesia.

Dr. Soetomo General-Academic Hospital, Surabaya, East Java, Indonesia.

出版信息

Radiol Case Rep. 2023 Oct 4;18(12):4370-4373. doi: 10.1016/j.radcr.2023.09.011. eCollection 2023 Dec.

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is one of the congenital cystic renal diseases with the highest incidence. ADPKD was suspected of being a risk factor for the emergence of RCC. A 65-year-old male complained of numbness in both knees for a week. The patient came to a neurosurgeon and was advised to perform a lumbosacral MRI. The patient had no complaints. The patient had a history of hypertension but was never treated. Computed tomography intravenous pyelogram (CT-IVP) revealed a heterogeneous lobulated mass in the upper to the middle of the right kidney to the right renal hilus. It also revealed multiple cysts, in both kidneys. The patient underwent an open radical nephrectomy in the right kidney with minimal bleeding. Three years revealed no pain at the surgery site or hematuria. Abdominal MRI revealed no recidive mass. This case report comprehensively described an autosomal dominant polycystic kidney disease that coexists with RCC. malignant lesions were found in ADPKD cases without any clinical symptoms of malignancy. M malignant lesions could be discovered by chance in nephrectomy specimens. Autosomal dominant polycystic kidney disease with renal cell carcinoma is a unique presentation. Despite the rarity of the situation, the patient was successfully treated.

摘要

常染色体显性多囊肾病(ADPKD)是发病率最高的先天性囊性肾病之一。ADPKD被怀疑是肾细胞癌(RCC)发生的一个危险因素。一名65岁男性主诉双膝麻木一周。该患者前往神经外科医生处就诊,医生建议进行腰骶部磁共振成像(MRI)检查。患者无其他不适主诉。患者有高血压病史,但从未接受过治疗。计算机断层扫描静脉肾盂造影(CT-IVP)显示右肾中上极至右肾门处有一个不均匀分叶状肿块。还显示双肾有多个囊肿。该患者接受了右肾开放性根治性肾切除术,术中出血极少。三年来手术部位无疼痛或血尿。腹部MRI显示无复发肿块。本病例报告全面描述了一例与RCC共存的常染色体显性多囊肾病。在无任何恶性临床症状的ADPKD病例中发现了恶性病变。恶性病变可能在肾切除标本中偶然发现。常染色体显性多囊肾病合并肾细胞癌是一种独特的表现。尽管这种情况罕见,但患者得到了成功治疗。

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