Objective: To report an unusual case of extrarenal nephroblastomatosis involving bilateral inguinal regions. Background: Nephroblastomatosis and nephrogenic rests are usually found in neonatal kidneys sub-clinically or associated with Wilms tumor. Extrarenal occurrence of this immature renal tissue is reported in literature as an incidental finding seen in inguinal canal, retroperitonium, along...

The WT1 gene, located at 11p13, encodes a zinc finger transcription factor involved in renal and gonadal development and in Wilms' tumor. Constitutional mutations of this gene have been described in most patients with Denys Drash syndrome (mesangial sclerosis associated with male pseudohermaphrodism and/or Wilms' tumor), but also in patients with genitourinary abnormalities and Wilms' tumor (WT...

Wilms tumor is a primarily sporadic disease, with only 1–2 % of affected individuals having a relative with Wilms tumor. However, bilateral Wilms tumors occur in approximately 5 % of cases, and Wilms tumor has been reported in association with more than 50 different genetic disorders, pointing to an underlying predisposition in further individuals. There is conclusive evidence of an increased r...

A 20-month-old female presented with a palpable abdominal mass. Imaging revealed bilateral renal masses and pulmonary nodules. The genetic evaluation identified de novo 540kb X-chromosome deletion involving GPC3, PHF6, HPRT1, highly skewed X-inactivation (100:0). This clinical picture was suggestive of Wilms’ tumor in the setting Simpson-Golabi-Behmel Syndrome.

We describe the case of a 5-year-old girl whose abdominal pain and distension were caused by Wilms tumor of the kidney. Because of the bilateral nature of her disease, she was spared biopsy or initial nephrectomy as part of her treatment course. Rather, she was treated presumptively for Wilms tumor based primarily on radiologic findings. Neoadjuvant chemotherapy consisting of vincristine, dacti...