Malignant rhabdoid tumour of the kidney occurring simultaneously with a brain tumour: a report of two cases and review of the literature

Abstract.

Malignant rhabdoid tumour of the kidney (MRTK), an uncommon aggressive neoplasm of children, is now recognised as a separate entity from Wilms' tumour with distinct clinical and pathological features. MRTK is unique in its significant association with primary brain tumours or brain metastases. We report two cases, aged 2 and 6 months, of MRTK occurring concurrently with a brain tumour. Radical nephrectomy and ventriculo-peritoneal shunting were performed. Both patients expired 2 and 6 months later despite receiving aggressive post-operative chemotherapy and radiotherapy. Conclusion: malignant rhabdoid tumour of the kidney is an uncommon neoplasm of early childhood with a poor prognosis. Due to its significant association with brain tumours or early brain metastases, concurrent brain computer tomographic examination is essential for all patients with this disease.