Nephrology- Structure, Congenital, Neoplasm & Trauma

Question 1

Wilms Tumor

Answer 1

Nephroblastoma arising from the metanephros and is one of the most common causes of malignant abdominal masses in young children.

WT1 gene

Question 2

Presentation of Wilms Tumor

Answer 2

Child 2-5
Unilateral Abd mass
Bilateral and earlier in WAGR

Abd pain
Hematuria
Hypertension

Question 3

Syndrome associated with Wilms tumor

Answer 3

WAGR
Beckwith-Wiedemann Syndrome
Denys-Drash syndrome

Question 4

WAGR syndrome

Answer 4

Wilms Tumor
Aniridia (absent irises)
Genitourinary abnormalities
Retardation (mental)

Question 5

Diagnosis of Wilms tumor

Answer 5

BIT: abdominal ultrasound.

CT/MRI show extent
Excision or biopsy are diagnostic

Question 6

Ddx wilms tumor and Neuroblastroma

Answer 6

Neuroblastoma: <2yo, arising neural crest sells (adrenal gland), possible HTN (less common)

Wilms: 2-5yo, arising from kidney, common HTN

Question 7

WAGR syndrome gene

Answer 7

gene deletion on chromosome 11p 13

Question 8

S&S Beckerwith-Weidemann

Answer 8

Wilms tumor
Hepatoblastoma
Neuroblastoma 
Hemihyperplasia
Macroglossia
Macrosomia