engleski

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

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

Down syndrome ; Wilms tumor

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