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患儿:女,1.5岁。以腹部巨大肿物1个月收入院。B 超示右腹膜后16 cm×12 cm×10 cm 混合性包块,左肾正常,右肾未能探及。肝功能及肾功能正常。IVU:右肾未显影,左肾正常。以腹膜后肿物行手术探查。术中见右腹膜后卵圆形包块,约16 cm×10 cm×9 cm 大小,质地中等,有完整包膜,其血供与右肾血管无关。右肾位于右髂窝,其肾包膜、皮质及输尿管正常。完整切除肿瘤,术后患儿恢复顺利。术后病理检查:肾母细胞瘤,以母细胞、上皮细胞为主,预后良好组织型。诊断为肾外肾母细胞瘤,NWTS Ⅰ期。采用长春新碱加更生霉素二联化疗,术后随访3年无复发。讨论:本病罕见,多数学者推测其来源于后肾或中肾异位残基,其他因素如细胞分化异常、胚胎多
Children: female, 1.5 years old. Large abdominal mass 1 month income hospital. B ultrasound showed right retroperitoneal 16 cm × 12 cm × 10 cm mixed mass, normal left kidney, right kidney failed to explore. Liver function and normal renal function. IVU: Right kidney is not developed, left kidney is normal. Surgical exploration of retroperitoneal tumor. Intraoperative see right retroperitoneal oval mass, about 16 cm × 10 cm × 9 cm in size, medium texture, a complete capsule, the blood supply and the right renal artery has nothing to do. Right kidney is located in the right iliac fossa, the renal capsule, cortex and ureter normal. Complete resection of the tumor, postoperative recovery of children. Postoperative pathological examination: Wilms tumor, mainly to the mother cells, epithelial cells, the prognosis of good tissue type. Diagnosis of extrarenal nephroblastoma, NWTS Ⅰ phase. The use of vincristine plus dactinomycin chemotherapy, no recurrence after 3 years of follow-up. Discussion: The disease is rare, most scholars speculate that it originates from the posterior kidney or kidney ectopic residues, other factors such as abnormal cell differentiation, embryos and more