Homsy Y L, Anderson J H, Oudjhane K, Russo P
Hopital Ste-Justine, University of Montreal, Canada.
J Urol. 1997 Dec;158(6):2256-9; discussion 2259-60. doi: 10.1016/s0022-5347(01)68227-2.
There is ongoing controversy concerning the management of multicystic dysplastic kidney disease, particularly with regard to the potential for malignant transformation. Our report fuels the debate by adding the 2 youngest patients in whom malignancy was present from birth or developed subsequently.
Two well documented cases of malignancy associated with multicystic dysplastic kidney disease are presented in 2 female infants (5 and 3 months old). The 5-month-old female infant was followed for multicystic dysplastic kidney disease and had no evidence of tumor either antenatally or at birth. The 3-month-old presented with hypertension and interventricular septal defect. A renal tumor was present on initial ultrasound.
Even though malignant degeneration is rare in multicystic dysplastic kidney disease, 9 cases have been reported in the literature so far. Of these cases 3 were Wilms tumor, 5 were renal cell carcinomas and 1 mesothelioma.
Our 2 cases lend support to the surgical management of multicystic dysplastic kidney disease, particularly as nephrectomy can now be performed in a day surgery setting with minimal morbidity. Only the risks of coexisting malignancy and possible malignant degeneration transformation are specifically addressed in this article. Other complications of multicystic dysplastic kidney disease such as hypertension, infection, abdominal pain, hematuria and persistent dysplastic renal tissue despite ultrasonographic resolution of multicystic dysplastic kidney disease are additional risk factors to be considered. A recommendation for nephrectomy in all cases of multicystic dysplastic kidney disease cannot be based only on these 2 cases. Several other factors must be weighed before making that decision.
关于多囊性发育不良性肾病的治疗存在持续争议，尤其是在恶性转化的可能性方面。我们的报告通过纳入2例出生时即存在恶性肿瘤或随后发生恶性肿瘤的最年幼患者，为这一争论增添了新内容。
报告了2例记录完整的与多囊性发育不良性肾病相关的恶性肿瘤病例，患者为2名女婴（分别为5个月和3个月大）。5个月大的女婴因多囊性发育不良性肾病接受随访，产前及出生时均未发现肿瘤迹象。3个月大的女婴表现为高血压和室间隔缺损，初次超声检查发现肾肿瘤。
尽管多囊性发育不良性肾病发生恶性退变很罕见，但迄今为止文献中已报道9例。其中3例为威尔姆斯瘤，5例为肾细胞癌，1例为间皮瘤。
我们的2例病例支持对多囊性发育不良性肾病采取手术治疗，特别是因为现在肾切除术可在日间手术环境下进行，发病率极低。本文仅特别讨论了并存恶性肿瘤和可能的恶性退变转化的风险。多囊性发育不良性肾病的其他并发症，如高血压、感染、腹痛、血尿以及尽管多囊性发育不良性肾病在超声检查中已消退但仍存在的发育不良肾组织，都是需要考虑的额外风险因素。不能仅基于这2例病例就建议对所有多囊性发育不良性肾病病例进行肾切除术。在做出该决定之前，必须权衡其他几个因素。
