Site Search:

Clear Cell Sarcoma Facts

• What is Clear Cell Sarcoma of the Kidney?
• What are symptoms of Clear Cell Sarcoma of the Kidney?
• What do we know about the biology of Clear Cell Sarcoma?
• How is Clear Cell Sarcoma diagnosed?
• How is Clear Cell Sarcoma treated?
• What is the prognosis for survival?
• Can Clear Cell Sarcoma come back?

What is Clear Cell Sarcoma of the Kidney?

Clear cell sarcoma of the kidney (CCSK) is a rare cancer, with only 20 new cases diagnosed each year in the United States. Although this is a rare cancer overall, it is the second most common kind of kidney cancer in children. The average age for the diagnosis of CCSK is 3 years old. Also, CCSK is extremely rare in infants younger than 6 months old and in children older than 14 years. There are no known risk factors for CCSK.

What are symptoms of Clear Cell Sarcoma of the Kidney?

• Fever
• Abdominal or flank mass
• Pain in the abdomen
• Blood in the urine
• High blood pressure

What do we know about the biology of Clear Cell Sarcoma?

Clear cell sarcoma is a rare disease and has not been studied systematically. Therefore, not much is known about the biology of this disease. However, to better understand CCSK, an open phase III clinical trial is now being done through the Children's Oncology Group. The goal of this trial is to create a tissue bank of tumor tissue, blood, and urine from each child with CCSK. These tissues are collected so that doctors can study them, gain a better understanding of the disease, and improve the treatment for this rare disease. Mainly doctors are trying to find out why some children respond better to treatment than others, so they can select the most specific treatment for each child. As part of this study, experts in clear cell sarcoma pathology and radiology review the tumor sample and imaging studies of each patient involved in the study. This will provide your child's doctor with a reliable resource for advice about your child's tumor

For more information about the clinical trials in Clear Cell Sarcoma.

How is Clear Cell Sarcoma diagnosed?

The first test used to diagnose clear cell sarcoma is often an abdominal ultrasound. A specialized ultrasound called a Doppler ultrasound is done to evaluate if the tumor has grown into the blood vessels. This is followed by a CT scan and/or MRI scan of the abdomen and pelvis to further evaluate the mass.

In addition, several imaging studies are used to make sure the tumor has not spread to other organs. A CT scan of the lung allows a doctor to see if the mass in the kidney has spread to the lung which is the most common site for this type of tumor to spread. Chest x-rays (CXR) are often done in addition to CT scans, and are used mostly during and after treatment. An MRI or CT scan of the brain is done to check and see whether or not the CCSK has spread to the brain. A bone scan and an x-ray skeletal survey should be done, since the bone is also a common site for CCSK to spread. A PET scan may be done, if it is available and if your child can hold still long enough for it.

In most cases, a surgeon will take out the tumor and the kidney after all of the imaging studies are done. If the imaging scans show that the tumor can not be removed, a biopsy must be done by taking a sample of the tumor tissue.

Once the diagnosis of clear cell sarcoma of the kidney has been confirmed by a pathologist, the oncologist will evaluate the results and assign a stage to the tumor. Staging means, assessing the characteristics of the tumor, whether it has spread, and if the surgeon was able to remove the entire tumor. The stage of the tumor will determine what type of treatment your child will have.

See how CCSK is staged.

How is Clear Cell Sarcoma treated?

Patients with clear cell sarcoma of the kidney are treated with the combination of surgery, chemotherapy, and radiation. The current recommendations are to remove the tumor first, then continue with chemotherapy and radiation. Tumors that can not be completely removed or that have spread to multiple places should be biopsied first. Following a biopsy of the tumor, chemotherapy is given to shrink the tumor. Re-evaluation for surgery is done at a later time.

• Treatment for patients with a removable tumor
• Treatment for patients with a non-removable tumor

What is the prognosis for survival?

As in most cancer, survival rates for clear cell sarcoma of the kidney depend on the stage of the tumor. Children with stage I disease have a > 97% survival, stage II > 75% survival, stage III > 77% survival, and stage IV > 50% survival. It is hoped that the newer therapies (such as Regimen UH1) for stage IV tumors will improve survival rates.

Can Clear Cell Sarcoma come back?

Clear cell sarcoma can come back. The most common sites for clear cell sarcoma of the kidney to come back are the bones, followed by the site of the original tumor. The tumor can come back after a prolonged period of time. Around 20% of the tumor recurrence occurs more than 3 years after diagnosis and some being as long as 10 years later. Therefore, prolonged observation is required and particular attention to signs of bone relapse are needed, such as watching for bone pain or a limp, and doing periodic bone scans.

Written and reviewed by: Susanne Ragg, MD, PhD; Deannie Lee, MD, PhD; Mary M Davis MD; James Hill, MD; Mercy Odueyungbo, B.S.; Ann Haddix, RN, MSN, CPNP; Jada Paine, MS; Julie Haydon, BS; Michelle Lucke, MPH; Elaine Skopelja, MALS, AHIP; Kellie Kaneshiro, AMLS, AHIP. Amber McMahon, B.S. Date last reviewed: August 2006.