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儿童单侧原发性肾肿瘤的保肾手术

Nephron-sparing surgery for unilateral primary renal tumor in children.

作者信息

Cozzi D A, Schiavetti A, Morini F, Castello M A, Cozzi F

机构信息

Università di Roma "La Sapienza," Policlinico Umberto I, Pediatric Surgery Unit, Rome, Italy.

出版信息

J Pediatr Surg. 2001 Feb;36(2):362-5. doi: 10.1053/jpsu.2001.20717.

Abstract

PURPOSE

Definition of the role of nephron-sparing surgery (NSS) in the treatment of children with primary unilateral renal tumor (URT).

METHODS

Between January 1992 and June 2000, 28 children with URT were admitted to our surgical unit. Criteria for selection of patients eligible for NSS were at least 50% of affected kidney preservable and stage I at surgery (frozen section biopsies from regional lymph nodes, perirenal fat, and surrounding renal parenchyma). Preoperative 2-drug chemotherapy was given to all patients more than 6 months of age. Between 1992 and 1995, 3-drug chemotherapy was used after NSS. Thereafter, following NSS, 2-drug chemotherapy was given if no microscopic residual disease was found on final histologic examination.

RESULTS

NSS was feasible in 10 of 28 children (35%). Enucleation of 6 tumors (1 metachronous) was performed in 5 patients. NSS was elective in 5 patients, mandatory in 3 patients (1 with aniridia and genitourinary anomalies, 1 with chronic glomerulonephritis, 1 with bilateral hyperplastic nephroblastomatosis), and advisable in 2 patients (1 with familial vesicoureteric reflux and 1 with cystic nephroma). Seven children had standard histology nephroblastoma, 1 highly differentiated epithelial type nephroblastoma, 1 oncocytoma, and 1 cystic nephroma. The only post-NSS complication was macroscopic hematuria in 1 patient. None of the patients had a relapse. All children are alive and disease free with good functioning of the affected kidney after NSS, at a mean follow-up of 40.7 months (range, 2 to 100 months).

CONCLUSION

NSS should be considered in selected children with URT, especially in patients with increased risk for metachronous tumor or renal disease, and in patients with benign or low-grade malignant URT.

摘要

目的

明确保留肾单位手术(NSS)在原发性单侧肾肿瘤(URT)患儿治疗中的作用。

方法

1992年1月至2000年6月,28例URT患儿入住我院外科。符合NSS条件的患者选择标准为患肾至少可保留50%且手术时为I期(区域淋巴结、肾周脂肪和周围肾实质的冰冻切片活检)。所有6个月以上的患者术前均接受两药化疗。1992年至1995年,NSS后使用三药化疗。此后,NSS后若最终组织学检查未发现微小残留病灶,则给予两药化疗。

结果

28例患儿中有10例(35%)可行NSS。5例患者对6个肿瘤(1个异时性肿瘤)进行了剜除术。5例患者NSS为选择性手术,3例为强制性手术(1例患有无虹膜和泌尿生殖系统异常,1例患有慢性肾小球肾炎,1例患有双侧增生性肾母细胞瘤),2例为建议性手术(1例患有家族性膀胱输尿管反流,1例患有囊性肾瘤)。7例患儿为标准组织学肾母细胞瘤,1例为高分化上皮型肾母细胞瘤,1例为嗜酸细胞瘤,1例为囊性肾瘤。NSS后唯一的并发症是1例患者出现肉眼血尿。所有患者均未复发。所有患儿均存活且无疾病,NSS后患肾功能良好,平均随访40.7个月(范围2至100个月)。

结论

对于选定的URT患儿应考虑行NSS,尤其是对于异时性肿瘤或肾脏疾病风险增加的患者,以及患有良性或低级别恶性URT的患者。

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