Wilms Tumor

Wilms Tumor:

Wilms tumor (WT), also known as nephroblastoma, is the most common primary malignant renal tumor of childhood.
It is the second most common malignant abdominal tumor in childhood.
The most common sites of metastases are the lungs, regional lymph nodes, and liver.
Histologically, the classic WT is made up of varying proportions of blastemal, stromal, and epithelial cells, recapitulating stages of normal renal development.
The treatment includes surgery and chemotherapy with or without radiotherapy.
The use of the current, multimodality treatment has dramatically improved the cure rate of WT from <30% to approximately 90% .
EPIDEMIOLOGY:
WT accounts for 6% of pediatric malignancies and more than 95% of kidney tumors in children. Approximately 75% of the cases occur in children younger than 5 yr with a peak incidence at 2-3 yr of age. It can arise in 1 or both kidneys; the incidence of bilateral WTs is 7%. Most cases are sporadic, but approximately 2% of patients have a family history. In 8-10% of patients, WT is observed in the context of hemihypertrophy, aniridia, genitourinary anomalies, and a variety of rare syndromes, including Beckwith- Wiedemann syndrome and Denys-Drash syndrome .An earlier age of diagnosis and an increased incidence of bilateral disease are generally observed in syndromic and familial cases.