Clear Cell Sarcoma Of Kidney C0334488
A rare, aggressive kidney cancer that primarily occurs in young children. Tends to spread to other areas, especially bones. Treated with surgery, chemotherapy, and sometimes radiation.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Gross hematuria
(urinary)Abdominal pain
(abdominal)Bone pain
(musculoskeletal)Anemia
(hematologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Radical Nephrectomy with Lymph Node Dissection
Complete surgical removal of the affected kidney along with regional lymph node dissection to remove the primary tumor and reduce metastatic risk. This is typically performed through an open or laparoscopic approach.
Multi-Agent Chemotherapy Protocol
Systemic chemotherapy using a combination of agents typically including vincristine, dactinomycin, doxorubicin, and cyclophosphamide to target residual microscopic disease and metastatic spread.
Additional treatment options
Radiation Therapy
Targeted radiation therapy to the tumor bed may be employed in cases with local recurrence risk or when surgical margins are uncertain. Modern techniques allow precise targeting while sparing healthy tissue.
procedureSurveillance Imaging Protocol
Regular imaging studies including chest CT, abdominal ultrasound, and MRI to monitor for disease recurrence or metastatic spread following initial treatment completion.
monitoringPsychosocial Support and Counseling
Comprehensive psychological support for the patient and family including counseling, support groups, and educational resources to address the emotional challenges of a pediatric cancer diagnosis and treatment.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Clear Cell Sarcoma Of Kidney C0334488?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
van den Heuvel-Eibrink MM, et al. Renal tumor classification and biology: the SIOP-RTSG experience. Pediatr Blood Cancer. 2022.
Gooskens SL, et al. Clear cell sarcoma of the kidney: a review. Eur J Cancer. 2019.
Kenny C, et al. BCOR-altered clear cell sarcoma of kidney: a distinct genetic subtype. J Pathol Clin Res. 2021.
Mendoza C, et al. Pediatric renal tumors: updates in surgical management. Urol Oncol. 2020.
Medication Comparisons
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy