Overview
Clear cell sarcoma of the kidney (CCSK) is a rare type of kidney cancer that occurs almost exclusively in young children. It is sometimes called "bone-metastasizing renal tumor of childhood" because it has a tendency to spread to the bones, which sets it apart from other childhood kidney tumors. CCSK accounts for roughly 3-5% of all childhood kidney tumors, making it much less common than Wilms tumor, the most frequent kidney cancer in children. CCSK typically presents as a mass in one kidney. The most common symptom is a painless lump or swelling in the abdomen that a parent or doctor may notice during a routine exam or bath time. Other symptoms can include blood in the urine, abdominal pain, fever, and high blood pressure. Because CCSK can spread to bones, lungs, brain, and soft tissues, children may also develop bone pain or other symptoms related to metastasis. Treatment usually involves surgery to remove the affected kidney (nephrectomy), followed by chemotherapy and sometimes radiation therapy. The treatment approach has improved significantly over the decades. With modern treatment protocols, including the use of the drug doxorubicin as part of combination chemotherapy, survival rates have improved considerably. However, CCSK is known for its potential to relapse even years after initial treatment, which requires long-term follow-up. Early diagnosis and aggressive treatment are key to achieving the best outcomes.
Also known as:
Key symptoms:
Painless lump or swelling in the abdomenBlood in the urineAbdominal pain or discomfortHigh blood pressureFever without an obvious causeBone pain (if cancer has spread to bones)Weight loss or poor appetiteFatigue or tirednessConstipation due to abdominal massNausea or vomiting
Sporadic
Usually appears on its own, not inherited from a parent
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Clear cell sarcoma of kidney.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Clear cell sarcoma of kidney at this time.
New trials open frequently. Follow this disease to get notified.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Clear cell sarcoma of kidney.
Community
No community posts yet. Be the first to share your experience with Clear cell sarcoma of kidney.
Start the conversation →Latest news about Clear cell sarcoma of kidney
No recent news articles for Clear cell sarcoma of kidney.
Follow this condition to be notified when news becomes available.
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What stage is my child's tumor, and has it spread to other parts of the body?,What treatment plan do you recommend, and what are the expected side effects?,How will we monitor for relapse after treatment is completed, and for how long?,What are the long-term effects of the chemotherapy and radiation on my child's health?,Are there any clinical trials available for clear cell sarcoma of the kidney?,What precautions should we take to protect my child's remaining kidney?,What support services are available for our family during and after treatment?
Common questions about Clear cell sarcoma of kidney
What is Clear cell sarcoma of kidney?
Clear cell sarcoma of the kidney (CCSK) is a rare type of kidney cancer that occurs almost exclusively in young children. It is sometimes called "bone-metastasizing renal tumor of childhood" because it has a tendency to spread to the bones, which sets it apart from other childhood kidney tumors. CCSK accounts for roughly 3-5% of all childhood kidney tumors, making it much less common than Wilms tumor, the most frequent kidney cancer in children. CCSK typically presents as a mass in one kidney. The most common symptom is a painless lump or swelling in the abdomen that a parent or doctor may no
How is Clear cell sarcoma of kidney inherited?
Clear cell sarcoma of kidney follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Clear cell sarcoma of kidney typically begin?
Typical onset of Clear cell sarcoma of kidney is childhood. Age of onset can vary across affected individuals.
Which specialists treat Clear cell sarcoma of kidney?
18 specialists and care centers treating Clear cell sarcoma of kidney are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.