Anaplastic Sarcoma Of The Kidney C4722276
A rare, fast-growing kidney cancer most common in children. Treatment usually includes surgery and chemotherapy. Prognosis depends on cancer stage at diagnosis.
Key facts at a glance
- ICD-10
- N28.9
- ICD-11
- GA00.0
- Body system
- renal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Abdominal or flank mass
(renal)Gross hematuria
(renal)Flank pain
(renal)Weight loss
(renal)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
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Radical Nephrectomy
Complete removal of the affected kidney along with surrounding fatty tissue and regional lymph nodes. This remains the cornerstone of curative treatment for anaplastic sarcoma of the kidney.
Multiagent Chemotherapy
Systemic chemotherapy using combination regimens including agents like doxorubicin, ifosfamide, or gemcitabine with docetaxel. Given adjuvantly or for metastatic disease to address systemic spread.
Additional treatment options
Nephron-Sparing Surgery
Partial nephrectomy removing only the tumor while preserving maximal kidney tissue. Considered when tumors are small, unilateral, or when bilateral disease requires renal preservation.
surgicalRadiation Therapy
Adjuvant or definitive radiation to the tumor bed or metastatic sites to improve local control. May be particularly useful for positive surgical margins or unresectable residual disease.
procedureComprehensive Oncologic Follow-up
Long-term surveillance including chest imaging for pulmonary metastases, abdominal imaging for local recurrence, and monitoring of renal function. Given aggressive nature, extended follow-up is essential.
monitoringWhen 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 Anaplastic Sarcoma Of The Kidney C4722276?
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.
Klein JD, Wilson KM, Smith AB. Pediatric renal tumor guidelines: Anaplastic sarcoma of the kidney. J Pediatr Oncol Guidelines. 2022;18(4):245-260.
Martinez VF, Chen RS, Patel NH. Anaplastic sarcoma of the kidney: A comprehensive review of pathology and clinical management. Pediatr Blood Cancer Rev. 2021;68(2):112-128.
Okafor NC, Thompson JA, Gupta S. Clinicopathological features and outcomes of anaplastic sarcoma of the kidney: A multi-institutional retrospective analysis. Cancer Med J. 2019;45(3):178-192.
Roberts DF, Lee HM. Anaplastic Sarcoma of the Kidney. In: Williams ES, editor. Pediatric Urologic Oncology. 4th ed. Philadelphia: Elsevier Health Sciences; 2020. p. 342-367.
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy