WILM’S TUMOUR
YASHWANTH N
ROLL NO 73
INTRODUCTION
• Wilms tumor (nephroblastoma) is the most common malignant tumor of
the kidney, accounting for 6-7% of all childhood malignancies.
• Eighty percent of patients with Wilms tumor present under 5 yr of age.
• The peak age at diagnosis is 2-3 y.
• 6% patients have bilateral disease.
• While a vast majority of Wilms tumors are sporadic, 1 -2% may be familial.
ETIOLOGY
• Nephrogenic rests may transform into Wilms tumors.(foci of embryonal
kidney tissue).
• WTl is the best characterized Wilms tumor gene. It is located at
chromosome llp13 and encodes for a transcription factor that is critical for
normal development of kidneys and gonads.
• WT2 is localized to a cluster of genes at llpl5.
• Loss ofheterozygosity (LOH) of lp or 16q and high expression of telomerase
have been associated with poorer outcome in children with Wilms tumor.
Children with some genetic syndromes are predisposed to developing Wilms
tumor.:
• WAGR Wilms tumor
• aniridia
• Genitourinary abnormalities like horseshoe or fused kidney
• mental retardation
• Deletion of llpl3
• Denys Drash syndrome
CLINICAL FEATURES
• Asymptomatic abdominal mass
• hematuria (10-25%)
• hypertension (25%)
• abdominal pain (30%)
• fever (20%)
• anorexia and vomiting
• Tumor thrombus extending into the inferior vena cava
• Other features include
• anemia
• thrombocytosis
• acquired deficiency of von Willebrand factor and factor VII
• polycythemia
DIFFERENTIAL DIAGNOSIS
• neuroblastoma
• hydronephrosis
• multicystic kidney
• rarely abdominal lymphoma
• retroperitoneal rhabdomyosarcoma
INVESTIGATION
STAGING OF TUMOUR
TREATMENT
• Therapy for Wilms tumor is based on stage of the disease and histology.
• Preoperative chemotherapy, using actinomycin D and vincristine for a period
of 4 weeks diminishes the size of the tumor and allows better staging.
• The immediate treatment for unilateral disease is removal of the affected
kidney.
• For stage 1 and 2 with favorable histology are usually treated postoperatively
with vincristine and actinomycin D.
• For advanced tumour combination of vincristine, actinomycin D and
adriamycin along with abdominal radiation.
PROGNOSIS
• Young age, low stage and low tumor weight ( <550 g) are favorable prognostic
factors.
• Focal anaplasia has better prognosis then diffuse anaplasia.
• Diploid tumors have a better prognosis than hyperdiploid tumors.
• Presence of anaplasia and loss of heterozygosity of lp or 16q increase risk of
recurrence.
• With modem therapy, 80-90% of patients with Wilms tumor are cured. Overall
approximately 90% of children with Wilms tumor are longterm survivors.
THANK YOU
