ICD10 code of Wilms Tumor and ICD9 code

The ICD-10 code for Wilms tumor, also known as nephroblastoma, is C64.1 (malignant neoplasm of the right kidney) or C64.2 (malignant neoplasm of the left kidney). Under the older ICD-9-CM classification system, Wilms tumor was coded as 189.0.

What is Wilms tumor?

Wilms tumor is the most common primary renal neoplasm of childhood, typically occurring in children between the ages of 2 and 5. It arises from primitive nephrogenic cells that fail to differentiate into normal kidney tissue. While Wilms tumor is generally considered a highly treatable malignancy with a survival rate exceeding 90% when managed with a multidisciplinary approach, it requires specialized pediatric oncology care.

How is Wilms tumor diagnosed and classified?

Diagnosis of Wilms tumor usually begins with imaging studies, such as abdominal ultrasound or CT scans, followed by a biopsy or definitive surgical resection. Pathologists classify Wilms tumor into two primary categories based on histology:

• Favorable Histology: The most common form, which shows a good response to standard chemotherapy and surgery.


• Anaplastic Histology: A rarer, more aggressive form characterized by nuclear abnormalities in the tumor cells, requiring more intensive treatment protocols.

Is there a genetic component to Wilms tumor?

While most cases of Wilms tumor are sporadic, approximately 1-2% of cases are familial. It is often associated with specific genetic syndromes, including WAGR syndrome, Denys-Drash syndrome, and Beckwith-Wiedemann syndrome. Genetic counseling is recommended for families where multiple members have been affected by Wilms tumor or when congenital anomalies are present.

Support for the Wilms tumor community

At DiseaseMaps.org, we currently support 18 individuals navigating the journey of Wilms tumor. Connecting with others who understand the unique stressors of pediatric oncology can provide essential emotional support for families during treatment and survivorship.

Next steps

• Consult a pediatric oncologist or a pediatric surgeon specializing in solid tumors.


• Request a referral to a genetic counselor if there is a family history of kidney abnormalities.


• Join the DiseaseMaps.org community to share experiences and find peer support.


• Review clinical trial eligibility through the Children’s Oncology Group (COG) if applicable.

Medical disclaimer: This content is for informational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician with any questions regarding a medical condition.

References

• National Cancer Institute (NCI) - Wilms Tumor and Other Childhood Kidney Tumors.


• NIH Genetic and Rare Diseases (GARD) Information Center - Wilms Tumor.


• Orphanet (ORPHA:91461) - Nephroblastoma.


• Children's Oncology Group (COG) - Guidelines for Renal Tumors.