Congenital mesoblastic nephroma (CMN) is a well-recognised renal tumour presenting in infancy, which has an excellent prognosis if completely excised. We describe the imaging appearances of an unusual, predominantly perinephric cystic CMN, with relative renal preservation but with retroperitoneal extension and bowel infiltration, which was complicated by hepatic metastases. To our knowledge, neither the appearance of the primary tumour nor the subsequent development of hepatic metastases has previously been reported. This appearance may represent a poor prognostic indicator for outcome. However, following partial hepatectomy, the patient remains disease-free at 1 year.
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