Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 2. The 2nd Decade: From the Radiologic Pathology...

Malignant renal tumors account for 7% of childhood cancers, and Wilms tumors are by far the most common-but not in older children and adolescents. Among individuals in the latter half of their 2nd decade of life, renal cell carcinoma (RCC) is more common than Wilms tumor. The histopathologic spectrum of RCCs in children differs from that in adults. The most common subtype of RCC in children and adolescents is Xp11.2 translocation RCC, which is distinguished by hyperattenuation at nonenhanced computed tomography, a defined capsule, and associated retroperitoneal lymphadenopathy. Papillary RCC is the second most common histologic subtype. It enhances less intensely compared with the adjacent renal parenchyma and has a propensity for calcification. Clear cell RCC is seen in patients with von Hippel-Lindau disease and is distinguished by its relatively hypervascular nature. Medullary carcinoma affects adolescents with the sickle cell trait and is characterized by an infiltrative growth pattern and extensive metastasis at presentation. Angiomyolipoma is seen in children with tuberous sclerosis complex and is often multifocal and hypervascular, with macroscopic fat. Metanephric tumors are central, circumscribed, and typically calcified. Lymphoma usually manifests as multifocal masses, but it may involve a solitary mass or infiltrative pattern. Extensive adenopathy and involvement of the gastrointestinal tract or other organs also may be seen. Primitive neuroectodermal tumor is an aggressive neoplasm that is typically quite large at diagnosis. Knowledge of the clinical, biologic, and histopathologic features of renal tumors in older children and adolescents and their effects on the imaging appearance can help the radiologist offer a useful preoperative differential diagnosis.