Which of the following statements regarding renal tumors of childhood and adolescence are true?

b. Clear cell sarcoma of the kidney has a high rate of metastasis to bone

c. Rhabdoid tumors may arise in the kidney, mediastinum or brain

Clear cell sarcoma of the kidney is presently considered a distinct histopathologic and clinical entity from Wilms’ tumor. It has a similar age distribution as that observed in Wilms’ tumor, but a markedly worsened prognosis. It is characterized by a proclivity to metastasize to bones and indeed has been called the bone metastasizing renal tumor of childhood. Relapse and death occur in 75% of patients with over half dying within one year of diagnosis. Aggressive systemic chemotherapy is recommended for all stages of the disease. Likewise, postoperative radiation to the tumor bed is recommended regardless of stage.

Rhabdoid tumors are rare malignancies that most commonly involve the kidney in childhood but may also occur primarily in the mediastinum or brain. Outcome is particularly poor and there is no proven chemotherapy regimen. Rhabdoid tumors of the kidney occur in infancy with a median age at presentation of 13 months. Survival rates are almost zero and even Stage I patients fare poorly. Aggressive therapy is warranted including surgical resection, local radiation therapy and systemic chemotherapy.