Wilms tumorDr.Hussein Alatabi
nephroblastomaEmbryonal malignancy of the kidney
Most cases are sporadic but 1-2% are familial
May arise in 1 or both kidneys
Associated with the following syndromes:
WAGR (aniridia, GU abnormalities, mental retardation)
Denys-Drash syndrome (early-onset renal failure, male pseudohermaphrodism)
Beckwith-Wiedemann syndrome (organomegaly, macroglossia, omphalocele, hemihypertrophy)
Epidemiology
2nd most common malignant abdominal tumor in childhoodIncidence is approximately 8 in 1 million children younger than age 15
About 500 new cases in North America per year
Peak incidence between ages 2 and 5
Individual with horseshoe kidney have twice the risk
Initial Presentation
Many children present with an asymptomatic abdominal mass1/3 have intermittent abdominal pain that may have been exacerbated by trauma
1/4 have gross or microscopic hematuria and is usually painless
1/4 have HTN
Systemic symptoms may occur, especially if there is bleeding into the tumor and associated anemia
About 10% will show pulmonary metastases at the time of diagnosis
Prognosis
Determined by tumor histology, stage, and molecular/genetic markers
Overall survival approaches 90% with either treatment protocol
Patients with diffuse anaplastic tumor have the worst prognosis
Approximately 15% with favorable histology and 50% with anaplastic features have tumor recurrent disease, most often involving the lung.
Treatment: Surgery,chemotherapy&radiotherapy