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Wilms' tumor

Wilms' tumor, also known as nephroblastoma, is a type of childhood kidney cancer that primarily a...

Wilms' tumor, also known as nephroblastoma, is a type of childhood kidney cancer that primarily affects young children, typically between the ages of 2 and 5 years old. It is the most common type of kidney cancer in children. Wilms' tumor usually occurs in just one kidney, although it can sometimes affect both kidneys.

Causes and Risk Factors:

  • The exact cause of Wilms' tumor is unknown, but it is thought to arise from genetic mutations that occur during fetal development.
  • Most cases of Wilms' tumor occur sporadically, without a known genetic predisposition. However, a small percentage of cases may be associated with genetic syndromes such as WAGR syndrome, Beckwith-Wiedemann syndrome, and Denys-Drash syndrome.

Symptoms:

  • Abdominal Mass: A firm, non-tender mass or swelling in the abdomen is the most common presenting symptom of Wilms' tumor.
  • Abdominal Pain: Some children may experience abdominal pain or discomfort.
  • Hematuria: Blood in the urine may be present, although it is less common.
  • Other Symptoms: Fever, loss of appetite, nausea, vomiting, and weight loss may occur in some cases.

Diagnosis:

  • Physical Examination: A healthcare provider may detect a palpable abdominal mass during a physical examination.
  • Imaging Studies: Ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI) scans are used to visualize the kidney and confirm the presence of a tumor.
  • Biopsy: A biopsy may be performed to obtain a tissue sample for microscopic examination and confirmation of the diagnosis. However, a biopsy is not always necessary before treatment is initiated, especially if imaging studies strongly suggest Wilms' tumor.

Treatment:

  • Surgery: Surgical removal of the tumor (nephrectomy) is the primary treatment for Wilms' tumor. Whenever possible, surgeons attempt to spare as much kidney tissue as possible to preserve kidney function.
  • Chemotherapy: Chemotherapy is typically administered before and after surgery to shrink the tumor, prevent metastasis (spread) of cancer cells, and eradicate any remaining cancer cells.
  • Radiation Therapy: Radiation therapy may be used in some cases, particularly if the tumor is large or if there is a high risk of recurrence.

Prognosis:

  • The prognosis for Wilms' tumor is generally favorable, especially when diagnosed and treated early.
  • The overall survival rate for children with Wilms' tumor is approximately 90%, with many children achieving long-term remission.
  • The prognosis may vary depending on factors such as the stage of the tumor, the extent of surgical resection, and the response to chemotherapy.

Follow-Up Care:

Regular follow-up visits with a pediatric oncologist are essential to monitor for recurrence of Wilms' tumor and to manage any potential long-term effects of treatment.

Supportive Care:

Children with Wilms' tumor and their families may benefit from psychological support, counseling, and access to support groups and resources.

Wilms' tumor is a rare but treatable form of childhood cancer. Prompt diagnosis, multimodal treatment approaches, and comprehensive supportive care contribute to favorable outcomes for the majority of children with Wilms' tumor.

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