Two siblings from one family presented with bilateral nephroblastoma. Both were treated by unilateral total nephrectomy and partial nephrectomy of the contralateral kidney and systemic chemotherapy. The older sibling is alive and has remained disease-free for 6-1/2 years from the date of diagnosis. The younger sibling is alive, diseasefree, and on postoperative chemotherapy. Both presented at the same age, which may shed some light on the genetic theory underlying the familial group. Review of the current literature is included.Wilms' tumor (WT) [12] is one of the most common intra-abdominal neoplasms found in children and accounts for approximately 7% of all neoplasms seen in children prior to puberty with a peak incidence during the third year of life. In 1814, the first recorded case of bilateral Wilms' tumor was recorded by Rance [10]. The incidence of bilateral cases in the literature varies from 4% to 10% of Wilms' tumor [7,13]. This incidence includes children who develop both tumors simultaneously (synchronous) and those who develop a second neoplasm following treatment of an apparent unilateral tumor (asynchronous WT). Most reported cases support the similarity between such cases and the two mutation models reported for retinoblastoma [14]. Utilizing experience of the National Wilms' Tumor Study Group (NWTS), the management concepts of bilateral simultaneous cases have been changed. It has been found that the aggressiveness of multi-modal therapy may be altered according to the stage as well as histology for maximal preservation of renal parenchyma [15] without adversely affecting prognosis. Case ReportsCase 1: This 19-month-old male presented 6-1/2 months previously with a left-sided abdominal swelling which had been diagnosed as Wilms' tumor and managed by left nephrectomy. He received a course of chemotherapy of actinomycin D and vincristine for three months. Six months later, ultrasonography showed a second lesion in the upper pole of the right kidney. This was confirmed by renal nuclear scan, diethylene triamine 5-acetic acid (DTPA), as well as computed tomography (CT) of the abdomen. Another course of chemotherapy resulted in shrinking the tumor size. Arteriography was performed prior to exploration and a right upper pole nephrectomy was performed successfully. The tumor histology was of mixed variety, epithelial predominant, favorable type, with negative lymph node sampling, placing it in group V of the total body staging according to NWTS classification. Postoperative chemotherapy was administered utilizing actinomycin D and vincristine for six months. No radiotherapy was administered. The patient is well and remains disease-free without evidence of recurrence of disease.Case 2: A 19-month-old male presented with an abdominal swelling. The patient's older brother presented with a similar problem at the same age six years ago.Clinical evaluation revealed a very large flank swelling extending anteriorly. Ultrasonography demonstrated a huge right solid renal mass occupying most of the right kidney ...
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.