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家族性肾母细胞瘤罕见。福清县医院和协和医院分别收治2例同胞兄妹(例1、2)和2例同胞兄弟(例3、4)肾母细胞瘤,现综合报告如下。例1 男,1.5月。全程肉眼血尿伴尿频、排尿啼哭6天,于1982年4月入院。体检:发育正常,心肺(一)。腹部略膨隆,左上腹可扪及18×14cm包块,质硬,表面光滑,随呼吸活动,无压痛。其他无明显异常。实验室检查:血沉60mm/h;尿常规:红细胞(+++),白细胞(+～++)。IVP:左肾未显影,右肾正常。在气管内麻醉下行左肾切除、左输尿管大部切除术。术中见左肾肿瘤占据肾中上部,仅下极可辨认出肾组织;肿瘤约20×17×14cm,表面光滑,包膜完整;输尿管增粗变硬。病理检查:光镜下见肿瘤主要由肉瘤样梭形细胞和未分化的后肾母细胞组成。病理诊断:左肾母细胞瘤。术后1年死亡。
Familial nephroblastoma rare. Fuqing County Hospital and Union Hospital were treated two siblings (cases 1,2) and two siblings (cases 3,4) Wilms tumor, the comprehensive report is as follows. Example 1 male, 1.5 months. The whole process of gross hematuria with frequent urination, urination crying 6 days, admitted to hospital in April 1982. Physical examination: normal development, cardiopulmonary (A). Abdomen slightly bulging, palpable left upper quadrant 18 × 14cm mass, hard, smooth surface, with respiratory activity, no tenderness. No other obvious abnormalities. Laboratory tests: ESR 60mm / h; urine routine: red blood cells (+++), white blood cells (+ ~ + +). IVP: left kidney is not developed, the right kidney is normal. In the endotracheal anesthesia underwent left nephrectomy, left ureteroidectomy. Surgery, see the left kidney tumors occupy the upper kidney, only the bottom can be identified under the kidney tissue; tumor about 20 × 17 × 14cm, smooth surface, complete capsule; ureteral thickening becomes harder. Pathological examination: The light microscope, see the tumor mainly by sarcomatoid spindle cells and undifferentiated renal cell after the composition. Pathological diagnosis: left nephroblastoma. 1 year after the death.