Signs and symptoms :

. Asymptomatic abdominal mass (in 80% of children at presentation)
. Abdominal pain or hematuria (25%)
. Urinary tract infection and varicocele (less common)
. Hypertension, gross hematuria, and fever (5-30%)
. Hypotension, anemia, and fever (from hemorrhage into the tumor; uncommon)
. Respiratory symptoms related to lung metastases (in patients with advanced disease; rare)

Diagnosis? Wilms tumor (nephroblastoma) is the most common childhood abdominal malignancy. The median age at diagnosis of Wilms tumor is approximately 3.5 years. With current multimodality therapy, approximately 80-90% of children with a diagnosis of Wilms Tumor.

The following lab studies are indicated:

. Chemistry profile – Including kidney function tests and routine measurements of electrolytes and calcium
. Urinalysis
. Coagulation studies
. Cytogenetics studies, including 1p and 16q deletion

Imaging studies are as follows:

. Renal ultrasonography (often the initial study)
. Four-field chest radiography (to detect lung metastases)
. Abdominal and chest CT
. Abdominal MRI
. Histologic confirmation with immediate nephrectomy, with exploration of the contralateral kidney to ensure that the disease is unilateral, and lymph node biopsy sampling for staging purposes. Immediate nephrectomy is not performed in patients with bilateral disease at presentation.

Management :

The usual approach is nephrectomy followed by chemotherapy, with or without postoperative radiotherapy. Chemotherapy regimens typically comprise vincristine and dactinomycin; doxorubicin and then cyclophosphamide and etoposide are added for increasingly high-risk disease. Children with loss of heterozygosity at 1p and 16q receive more aggressive chemotherapy.

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