Conlon Niamh, Teoh Chia Wei, Pears Jane, O'Sullivan Maureen
Cellular Pathology Department, AMNCH, Tallaght, Dublin, Ireland.
BMJ Case Rep. 2012 Sep 7;2012:bcr0220125854. doi: 10.1136/bcr.02.2012.5854.
The authors describe the case of a 16-month-old girl who presented with bilateral hydronephrosis and renal failure secondary to bilateral renal pelvic botryoid Wilms' tumour (nephroblastoma). The term 'botryoid' describes an intrapelvic polypoid renal Wilms tumour, either extending from the renal parenchyma or primarily pelvis-based tumour. Both tumours filled the renal pelvis and extended down the proximal ureter, with no intraparenchymal tumour seen. Bilateral intralobar nephrogenic rests were present. Histological examination demonstrated triphasic nephroblastoma, with focal rhabdomyomatous differentiation of the stromal element bilaterally. Postchemotherapy, the patient underwent bilateral nephrectomy. Post complete resection of her bilateral disease, this patient has had an excellent outcome. This is only the third reported case of bilateral botryoid Wilms' tumour. While the condition is extremely rare, it should be included in the differential diagnosis of a young child with bilateral renal pelvic masses.
作者描述了一名16个月大女孩的病例,该女孩因双侧肾盂葡萄状威尔姆斯瘤(肾母细胞瘤)继发双侧肾积水和肾衰竭。术语“葡萄状”描述的是盆腔内息肉样肾威尔姆斯瘤,要么从肾实质延伸,要么主要是基于盆腔的肿瘤。两个肿瘤都充满肾盂并延伸至近端输尿管,未发现肾实质内肿瘤。双侧存在叶内肾源性残留。组织学检查显示为三相肾母细胞瘤,双侧间质成分有局灶性横纹肌样分化。化疗后,患者接受了双侧肾切除术。在双侧疾病完全切除后,该患者预后良好。这是第三例报告的双侧葡萄状威尔姆斯瘤病例。虽然这种情况极为罕见,但在患有双侧肾盂肿块的幼儿鉴别诊断中应予以考虑。