Summary
Highlights
Wilms tumor, also known as nephroblastoma, is a kidney cancer primarily affecting children under five years old. It accounts for about 6% of all cancers in children under 15, with a 90% survival rate.
Wilms tumor is caused by a chromosomal deletion of the Wilms Tumor 1 (WT1) suppressor gene, located on chromosome 11. Approximately 10% of patients may also have WAGR syndrome (Wilms tumor, Aniridia, Genitourinary abnormalities, and mental Retardation).
Key symptoms include a painless abdominal mass, hematuria (blood in urine), and hypertension. Diagnosis involves abdominal imaging (ultrasound or CT) and a biopsy, which reveals characteristic elements of bone, muscle, and cartilage. Treatment typically combines surgery and chemotherapy, with radiation therapy being an additional option.
A 13-month-old boy presents with decreased appetite, reduced activity, and a large, firm abdominal mass on the left side, consistent with Wilms tumor. Other conditions like splenic rupture, Hodgkin disease, leukemic infiltration, and Meckel's diverticulum are ruled out based on the symptoms and lab results.
A two-year-old child presents with hematuria, hypertension, and an abdominal mass. Biopsy confirms Wilms tumor with characteristic stromal components. The question focuses on identifying the chromosome where the gene for this disorder is localized, which is chromosome 11.