CAJ | 학술논문

目的评估双侧肾母细胞瘤的诊治及长期存活质量.方法回顾性分析1997～2010年收治的肾母细胞瘤315例,其中双侧11例(3.5％)；8例同期性(synchronous),3例不同期性(meta-chronous)均于一侧瘤肾切除后1～2.5年经B超检出.男7例,女4例.同期性平均年龄:17月龄；不同期者第二次肿瘤被检出年龄分别为4岁、4岁3月龄及20月龄.并发畸形中有1例左睾缺如、1例右睾下降不全、1例尿道下裂Ⅲ度,1例双侧虹膜缺如.11例的病理组织均属良好型；同期性中1例双侧均有肾母细胞瘤病；另1例右侧有肾母细胞瘤病.术前均接受化疗,最少4周,长春新碱(VCR)或加放线菌素D(ACTD).手术以肿瘤分期剜除为主,术后用VCR+ ACTD+ ADR(阿霉素)15个月,定期随诊最少5年.结果 8例随访20个月至14年(平均4.2年)无瘤健康存活,1例不同期者失访,1例做右瘤肾切除、同期探查左肾门肿瘤,术后无尿死亡.1例11个月女婴死于肿瘤复发.结论双侧肾母细胞瘤就诊时患儿年龄小,病理组织多属良好型,术前化疗使肿瘤缩小,便于完整挖除,保留肾单位.伴随的畸形和肾母细胞瘤病并未影响预后.
목적 평고쌍측신모세포류적진치급장기존활질량.방법 회고성분석1997～2010년수치적신모세포류315례,기중쌍측11례(3.5％)；8례동기성(synchronous),3례불동기성(meta-chronous)균우일측류신절제후1～2.5년경B초검출.남7례,녀4례.동기성평균년령:17월령；불동기자제이차종류피검출년령분별위4세、4세3월령급20월령.병발기형중유1례좌고결여、1례우고하강불전、1례뇨도하렬Ⅲ도,1례쌍측홍막결여.11례적병리조직균속량호형；동기성중1례쌍측균유신모세포류병；령1례우측유신모세포류병.술전균접수화료,최소4주,장춘신감(VCR)혹가방선균소D(ACTD).수술이종류분기완제위주,술후용VCR+ ACTD+ ADR(아매소)15개월,정기수진최소5년.결과 8례수방20개월지14년(평균4.2년)무류건강존활,1례불동기자실방,1례주우류신절제、동기탐사좌신문종류,술후무뇨사망.1례11개월녀영사우종류복발.결론 쌍측신모세포류취진시환인년령소,병리조직다속량호형,술전화료사종류축소,편우완정알제,보류신단위.반수적기형화신모세포류병병미영향예후.
Objective To evaluate the management and therapeutic efficacy of bilateral Wilms tumor. Methods From 1997 to 2010, total 315 patients with Wilms tumor were treated in our hospital and 11 cases (3.5％) of them were bilateral, including 8 cases synchronous tumor and metachronous tumor in 3 cases. The mean age in synchronous cases during primary diagnosis was 17 months (ranging 10 months to 2 years) ; the age in metachronous cases was respectively 4 years, 4 years and 3 months, and 20 months. The metachronous tumor was found via ultrasonography in follow up period after unilateral nephrectomy. There were 7 boys and 4 girls in all cases. 4 children had associated congenital abnormalities, including absent of left testis in 1 case, incompletely descended right testis in 1 case, and hypospadias in 1 case, as well as aniridia in 1 case. All 11 cases were favorable histological features. In synchronous cases, one associated with bilateral and one with right sided nephroblastomosis. All the patients underwent pre-operative chemotherapy (vincristine-VCR dactinomycin D-ACTD)for at least 4 weeks. Staged bilateral tumor enucleation was the main procedure. After surgery VCR +ACTD + adriamycin protocol were conducted for 15 months. Follow-up time was more than 5 years. Results 8 children were followed-up for average 4.2 years (ranging 20 months to 14 years) and they were alive and healthy. One metachronous was loss to follow-up. one child received right sided nephrectomy and at the mean time exploration on the left side, died of anuria postoperatively. 1 case (female, 11 months old) died of tumor recurrence. Conclusions The age of patient with bilateral Wilms tumor was more younger. Majority of them has favorable histologic features. Preoperative chemotherapy is an effective way to reduce tumor volume before staged procedure as it can help to preserve functional renal tissue. The associated congenital abnormalities and nephroblastomosis seem not increased complications.