Clinical analysis of six cases of nephroblastomatosis combined with Wilm's tumor
10.3760/cma.j.issn.1000-6702.2011.05.007
- VernacularTitle:肾母细胞瘤合并肾母细胞瘤病6例临床分析
- Author:
Hongcheng SONG
;
Ning SUN
;
Weiping ZHANG
;
Jiwu BAI
;
Jun TIAN
;
Xianghui XIE
;
Minglei LI
;
Ning LI
;
Chengru HUANG
- Publication Type:Journal Article
- Keywords:
Nephroblastomatosis;
Wilm's tumor;
Chemotherapy;
Operation
- From:
Chinese Journal of Urology
2011;32(5):313-315
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the diagnosis,treatment and prognosis of nephroblastomatosis (Nbm) combined with Wilms'tumor (WT). Methods Clinical data of six patients treated for WT combined with Nbm in Beijing Children's Hospital from 2006 to 2010 were reviewed retrospectively.The patients'ages ranged from five to 14 months.Two of the patients were female and four were male.The WTs were left-sided in four cases and right-sided in two cases.The Nbms were ipsilateral with WT in three cases,contralateral in two cases and bilateral in one case. The Nbms were single In three cases and multiple in three cases.WTs were all single and the maximum diameter was 3,4,8,10,11,and 12 cm respectively.Two paitents underwent nephrectomy.Nephron sparing surgery and upper and lower pole nodule biopsy was conducted in two cases,Nephrectomy and contralateral nephron sparing surgery was conducted in an additional two cases.Adjuvant chemotherapy included vincristine,actinomycin and doxorubicin. Results One patient had tumor recurrence 33 months after a 15 month regimen of postoperative chemotherapy. One patient had tumor recurrence and died after nephron sparing surgery 5 months after a 11 month regimen of chemotherapy.Four patients underwent 6 months of chemotherapy,and it was 9,12,and 21 months respectively after stop of chemotherapy.Another patient was still in chemotherapy. Conclusions Nbm is a pre-neoplastic proliferative process with high risk of developing WT.Chemotherapy may reduce the rate of Nbm malignancy.If Nbm is malignant or chemotherapy is invalid,nephron sparing surgery is recommended.
