Wilms Tumor

Wilms tumor is a rare kidney cancer that primarily affects children, usually under the age of five, and is also known as nephroblastoma. It arises from immature kidney cells and is the most common type of pediatric renal cancer. The tumor often presents as a painless abdominal mass discovered by a caregiver or during a routine examination. Other symptoms may include abdominal pain, blood in the urine, fever, hypertension, or constipation. Wilms tumor is typically unilateral but may occur bilaterally in some cases, especially in children with genetic syndromes such as WAGR syndrome, Denys-Drash syndrome, or Beckwith-Wiedemann syndrome. Diagnosis is confirmed through imaging techniques like ultrasound, computed tomography, or magnetic resonance imaging, followed by surgical exploration and histological confirmation. The tumor is staged based on its spread, and this staging guides treatment planning. Most cases are treated with a combination of surgery, chemotherapy, and, in some instances, radiation therapy. The standard approach involves nephrectomy to remove the affected kidney, followed by chemotherapy to target residual cancer cells. For bilateral tumors or tumors associated with genetic conditions, kidney-sparing surgeries may be considered. Prognosis is generally favorable, with survival rates exceeding 90 percent in localized disease, due to well-established treatment protocols developed through international cooperative studies. However, treatment side effects, including potential damage to remaining kidney tissue, infertility, and cardiac toxicity from certain chemotherapy drugs, must be carefully managed. Long-term monitoring is essential to detect any recurrence and to address late effects of therapy. Advances in genetic research have identified molecular alterations such as WT1 and WT2 gene mutations, which have improved understanding of tumor development and risk stratification. Efforts are ongoing to personalize therapy further by reducing treatment intensity in low-risk patients while intensifying it for those with high-risk features. Wilms tumor remains a success story in pediatric oncology due to the high cure rates achieved through global collaboration, but challenges persist in ensuring timely diagnosis, access to care, and supportive services, especially in low-resource settings. Multidisciplinary care involving pediatric oncologists, nephrologists, surgeons, radiologists, and psychologists is crucial to support the medical and emotional well-being of both patients and their families throughout treatment and survivorship.

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