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.
ABSTRACT
In contrast to the nodular renal blastema which is defined by nests of primitive metanephric cells after 36 weeks of gestation, the nephroblastomatosis is characterized by neoplastic proliferation of the primitive cells. This lesion is presumed to be closely related to the development of Wilms' tumor. We report a case of bilateral nephroblastomatosis associated with Wilms' tumor in a child. This 4 1/2 year-old girl was admitted because of a 10 cm-sized round mass in the right kidney, and smaller nodules in the left kidney and the lung. After three cycles of chemotherapy and subsequent disappearance of the nodules in the left kidney and lung, she underwent a right nephrectomy and a wedge resection of the left kidney. A round Wilms' tumor mass was seen in the lower pole of the right kidney. Remaining right renal cortex showed multiple, slightly depressed gray-white nodules associated with multiple samll cysts. They were comprised of multifocal subcapsular nests of primitive nephrogenic cells with focal tubular or glomerular differentiation. They resembled fetal renal tissue. In the left kidney, similar nests of primitive cells were also noted. These lesions were interpreted as multifocal perilobar type of nephroblastomatosis.