DOI: 10.363247/sjmcr.2018.v06i10.035

Pages: 874-880

The nephroblastomatosis is defined by the presence of multiple or diffuse nephrogenic remains, regarded as precursor’s nephroblastoma and appear in up to 90% of bilateral Wilms, nephroblastoma is the first renal tumor of the child. Our work is a retrospective study of 2 cases of bilateral Wilms and nephroblastomatosis. The average age was 20 months, both are female. The circumstances of discovery were the finding of abdominal distention, fever or hematuria with Beckwith-Wiedmann diagnosed syndrome. At the end of the assessment, particularly by ultrasound and abdominal CT scan diagnosis in two cases of bilateral Wilms tumor non metastatic and nephroblastomatosis is made. The treatment consisted of preoperative chemotherapy, the former having responded well to chemotherapy then secondary tumor progressed, that led to an extended unilateral nephrectomy followed by chemotherapy and radiation therapy postoperatively. The second case has also responded well to chemotherapy currently stands tumor progression with paraspinal extension thus changing the treatment with chemotherapy nephroblastoma at high risk. At the end of this work and in the light of the most recent data from the literature, we conclude that the nephroblastomatosis precursor nephroblastoma including their genetic and pathological characteristics similar making it difficult to differentiate, medical imaging of bilateral Wilms and nephroblastomatosis plays a role primary diagnosis but cannot differentiate between the two. Therapeutically, the treatment strategy is to prolonged preoperative chemotherapy according to response to chemotherapy followed by unilateral nephrectomy with a postoperative treatment adapted to the local tumor stage.