Ngan K W, Shaari S, Subramaniam T
Department of Pathology, Serdang Hospital, Jalan Puchong, Kajang 43000, Malaysia.
Singapore Med J. 2009 Sep;50(9):e329-31.
We report a six-year-old Malay girl who presented with a right retroperitoneal tumour that measured 7.5 cm in diameter. A wide excision of the lesion was performed. The tumour was separated from a normal-appearing right kidney by a capsule. Microscopically, this well-encapsulated tumour was composed of classical triphasic components: epithelial, mesenchymal and blastemal areas. The immunohistochemical study showed WT1 expression in the blastemal area. Thus, a diagnosis of Wilms' tumour with favourable histology was made. The patient was well for 12 months. Extrarenal Wilms' tumour is uncommon, with the majority of cases occurring in the retroperitoneal and inguinal areas. Wilms' tumour that is not arising from the intrarenal area but shares a common capsule with the ipsilateral kidney, is even rarer. Indeed, our case would be more appropriately classified as juxtarenal/pararenal Wilms' tumour. Despite its rarity, an extrarenal or even juxtarenal/pararenal Wilms' tumour should be included in the differential diagnosis of retroperitoneal tumour.
我们报告了一名6岁的马来女孩，她患有一个直径为7.5厘米的右腹膜后肿瘤。对该病变进行了广泛切除。肿瘤通过一个包膜与外观正常的右肾分离。显微镜下，这个包膜完整的肿瘤由典型的三相成分组成：上皮、间充质和胚基区域。免疫组织化学研究显示胚基区域有WT1表达。因此，诊断为组织学良好的肾母细胞瘤。患者病情稳定12个月。肾外肾母细胞瘤并不常见，大多数病例发生在腹膜后和腹股沟区域。并非起源于肾内区域但与同侧肾脏共用一个包膜 的肾母细胞瘤更为罕见。实际上，我们的病例更适合归类为肾旁/肾周肾母细胞瘤。尽管其罕见，但肾外甚至肾旁/肾周肾母细胞瘤应纳入腹膜后肿瘤的鉴别诊断。
