Thummala Yashaswi, Parmar Kalpesh, Mathew Jeni, Tyagi Shantanu, Kumar Santosh
Department of Urology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.
J Endourol Case Rep. 2020 Dec 29;6(4):353-357. doi: 10.1089/cren.2020.0104. eCollection 2020.
Autosomal dominant polycystic kidney disease (ADPKD) is most common potentially lethal cystic disease occurring in ∼1 in 1000 live births. It is an important cause of end-stage renal disease, which occurs in 75% of patients by the age of 70 years. APDPKD is a systemic disease with involvement of multiple extrarenal organs. Incidence of renal cell cancer in ADPKD is no more than in normal population. High index of suspicion is required due to gross distortion of renal architecture. We report a 56-year male, known case of ADPKD on maintenance hemodialysis presenting with hematuria. On evaluation, he was diagnosed with bilateral renal masses on contrast imaging. Bilateral laparoscopic nephrectomy was performed and specimen was retrieved from pfannenstiel incision. Histology showed papillary renal cancer in left kidney and oncocytoma in right kidney with negative margins. Minimally invasive surgery in ADPKD with renal mass is challenging due to space constraints and large size kidneys. However, laparoscopic approach is a feasible option with minimal morbidity, less pain, and speedy recovery, specially in chronic kidney disease patients already immunocompromised status.
常染色体显性多囊肾病（ADPKD）是最常见的潜在致命性囊性疾病，在每1000例活产中约有1例发生。它是终末期肾病的重要病因，75%的患者在70岁时会发展至此阶段。ADPKD是一种累及多个肾外器官的全身性疾病。ADPKD患者患肾细胞癌的发生率并不高于正常人群。由于肾脏结构严重变形，需要高度怀疑。我们报告一例56岁男性，已知患有ADPKD，正在接受维持性血液透析，出现血尿。经评估，对比成像显示他双侧肾脏有肿块。进行了双侧腹腔镜肾切除术，并通过耻骨上横切口取出标本。组织学检查显示左肾为乳头状肾癌，右肾为嗜酸细胞瘤，切缘阴性。由于空间受限和肾脏体积较大，ADPKD合并肾肿块时进行微创手术具有挑战性。然而，腹腔镜手术是一种可行的选择，发病率最低，疼痛减轻，恢复迅速，特别是对于已经处于免疫功能低下状态的慢性肾病患者。
