Kälble T, Staehler G, Richter G M, Waldherr R
Urologische Abteilung, Chirurgischen Universitäts-Klinik Heidelberg.
Urologe A. 1990 Jul;29(4):215-8.
We report on a 42-year-old patient with a Wilms tumour (nephroblastoma) of the right kidney, which was detected incidentally by sonography. Both radiological examination and intraoperative examination of a quick-frozen section led to a diagnosis of malignant renal tumour. Post-operative histological examination showed a nephroblastoma of predominantly epithelial cell type with no signs of anaplasia. Only about 210 cases of Wilms tumours in adults have been reported in the world literature so far. Because of this low incidence and the frequent lack of any clear distinction from sarcomatoid renal cell carcinoma in terminology and histology, no standardized therapy regimens exist. The prognosis with a 3-year survival rate of 24%-44% is poor compared with that of Wilms tumours in children. In a literature review the current status of knowledge about nephroblastomas in adults is discussed.
我们报告了一名42岁患有右肾威尔姆斯瘤(肾母细胞瘤)的患者,该肿瘤通过超声检查偶然发现。放射学检查和术中快速冰冻切片检查均诊断为恶性肾肿瘤。术后组织学检查显示主要为上皮细胞型的肾母细胞瘤,无间变迹象。迄今为止,世界文献中仅报道了约210例成人威尔姆斯瘤病例。由于发病率低,且在术语和组织学上常与肉瘤样肾细胞癌难以明确区分,因此不存在标准化的治疗方案。与儿童威尔姆斯瘤相比,其3年生存率为24%-44%,预后较差。本文献综述讨论了目前关于成人肾母细胞瘤的知识现状。