Wilms Tumor

Description

Epidemiology Most common malignant renal tumor in childhood Peak: 3-4 years Cause: Sporadic Hereditary Genetic disorder Female > male Genetic WT1 – tumor suppressor gene - chromosome 11p12 - likely specific role in glomerular & gonadal development - Denys Drash syndrome - germline mutations are seen in 82% of WT patients with GU anomalies or renal failure - sporadic WT = 20% - familial WT = 4% Clinical syndrome 1. WAGR Syndrome - Wilms tumor, Aniridia, Genitourinary malformations, mental Retardation 2. Denys –Drash Syndrome - Wilms tumor, pseudohermaphroditism, mesangial sclerosis, renal failure 3. Beckwith-Wiedemann Syndrome - Somatic gigantism, omphalocele, macroglossia, GU anomalies, ear creases, hypoglycemia, hemihypertrophy, WT & other malignancies predisposition 4. Simpson-Golabi-Behmel Syndrome

Objectives

Presenters

1. Dr. Vivian Gathecha
   Dr.