Clear-cell sarcoma of the kidney

Clear-cell sarcoma of the kidney (CCSK) is a rare type of kidney cancer that primarily affects children. It is characterized by its unique histological appearance, which is distinct from other pediatric renal tumors such as Wilms' tumor and renal cell carcinoma.

Epidemiology

CCSK accounts for approximately 2-4% of all pediatric renal tumors. It is most commonly diagnosed in children aged 2-4 years, but can occur at any age. The incidence is slightly higher in males than in females.

Pathogenesis

The exact cause of CCSK is unknown. However, it is believed to originate from the metanephric mesenchyme, the embryonic tissue that gives rise to the kidneys. Some cases have been associated with certain genetic abnormalities, such as the t(10;17)(q22;p13) translocation.

Clinical Features

Children with CCSK often present with a large, painless abdominal mass. Other symptoms may include hematuria (blood in the urine), hypertension (high blood pressure), and anemia (low red blood cell count). In some cases, the tumor may spread to other parts of the body, most commonly the lungs and bones.

Diagnosis

The diagnosis of CCSK is typically made based on the results of imaging studies, such as ultrasound or computed tomography (CT) scan, and confirmed by biopsy of the tumor. The tumor has a characteristic appearance under the microscope, with clear cells arranged in nests or cords.

Treatment

The standard treatment for CCSK includes surgery to remove the tumor, followed by chemotherapy and/or radiation therapy. The choice of treatment depends on the stage of the disease and the patient's overall health.

Prognosis

The prognosis for children with CCSK is generally good, with a 5-year survival rate of approximately 80-90%. However, the prognosis can be significantly worse for children with advanced disease or those who experience a recurrence.

See Also

• Kidney cancer
• Pediatric oncology
• Wilms' tumor
• Renal cell carcinoma