Coexistence of synchronous bilateral Wilms tumor and trisomy 21 – first report and review of literature

The bilateral form of the Wilms tumor appears only in 5% of cases, and the incidence in children under the age of 15 is about 1: 250,000. Contrary to the expectations, large population-based studies have shown that the incidence of Wilms tumor is much lower in the population with trisomy 21 than in the general population. To our knowledge, this is the first reported case of synchronous bilateral Wilms tumor appearing in a patient with trisomy 21. Case report: A 19-month-old male, previously known for trisomy 21, was admitted to hospital because of chronic constipation and abdominal pain. A month before the child started to cry and suffer pain during defecation. He had the last stool seven days before the examination. Abdominal palpation in the left hypochondriac region revealed a solid mass of about 6x6 cm in size. Ultrasound and Multislice Computed Tomography showed a tumor on both kidneys; the right was smaller, and the left was larger -destroying most of the parenchyma and causing the bowel obstruction. After 3 weeks of chemotherapy, the radical left nephrectomy and the partial right nephrectomy were made. 28-week chemotherapy continued postoperatively. Histopathology tests confirmed the diagnosis of Wilms tumor. After recovery, the patient had a sufficient renal function. Conclusion: The coventional treatment with a combination of chemotherapy and surgical resection showed a good long-term outcome. However, in these cases a special caution should be focused on the quantity of preserved renal tissue, because of an increased risk of renal failure.