Renal Clear Cell Sarcoma

Synonyms

Bone metastasizing renal tumor of childhood

Definition

Clear cell sarcoma of the kidney is a rare malignant mesenchymal tumor occurring in children.

Clinical Features

• Incidence

  Clear cell sarcoma comprises 4% of pediatric renal tumors.

• Age

  It occurs usually at 2–3 years of age and it is extremely rare before 6 months.

• Sex

  Male-to-female ratio is 1.6:1.

• Site

  The tumor usually shows the epicenter at the renal medulla.

• Treatment

  The treatment consists of surgical resection, when possible, followed by chemotherapy and radiotherapy.

• Outcome

  With current treatment (addition of doxorubicin to chemotherapeutic protocols), outcome has improved with a 5-year overall survival of 75–90%. Bone metastases are common, and unusual sites, such as soft tissues of orbit, may occur. Brain metastases are more common, due to blood-brain barrier which impedes effective chemotherapy. True stage I disease has a favorable prognosis, and chemotherapy protocols are stage-dependent (Argani et al. 2000).

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