10.30: Renal V

Question 1

What does the metanephric blastema develop into?

Answer 1

Nephrons

Question 2

What does the ureteric bud develop into?

Answer 2

Collecting duct system

Question 3

Are bilateral or unilateral lesions genetic?

Answer 3

Bilateral, unilateral cannot be genetic

Question 4

What is agenesis?

Answer 4

Absence of something

Question 5

What occurs in horseshoe kidneys?

Answer 5

They are fused

Question 6

What is dysplasia?

Answer 6

Abnormal development

Question 7

What is polycystic?

Answer 7

Cystic but without dysplastic elements

Question 8

What is hypoplasia?

Answer 8

Small but no dysplasia demonstrated

Question 9

What are enlarged irregular kidneys appearing like bunch of grapes characteristic of?

Answer 9

Cystic renal dysplasia

Question 10

What is oligohydramnios?

Answer 10

• Lack of amniotic fluid due to agenesis of kidneys
• Urine is responsible for amniotic fluid
• Amniotic fluid responsible for development of lungs
• Leads to hypoplastic lungs
  “Potters syndrome”

Question 11

What is potters syndrome?

Answer 11

Hypoplastic lungs due to agenesis of kidneys

Question 12

What is ARPKD?

Answer 12

Childhood “Autosomal recessive polycystic kidney disease”

• Enlarged kidneys with retained shape
• Liver cysts seen as well

Question 13

What is APKD?

Answer 13

“Adult polycystic Kidney disease”

• More common than childhood recessive
• Autosomal dominant
• Huge kidney with cysts in liver as well
• Nephrons between cysts are functioning

Question 14

Treatment of APKD?

Answer 14

• Transplant

Question 15

What is often associated with APKD?

Answer 15

1. Hepatic cysts
2. Intracranial berry aneurysms
3. Mitral prolapse

Question 16

3 types of cystic disease?

Answer 16

1. Developmental dysplasia
2. APKD
3. ARPKD

Question 17

Only cystic disease without liver abnormalities?

Answer 17

Dysplasia

Question 18

Only cystic disease with ureter abnormalities?

Answer 18

Dysplasia

Question 19

Only cystic disease without reniform shape?

Answer 19

Dysplasia

Question 20

What is acquired cystic disease associated with?

Answer 20

• Dialysis

- High rate of developing renal carcinoma

Question 21

Which cystic disease has high risk of renal carcinoma?

Answer 21

Acquired

Question 22

Which kidney tumors are presumed malignant?

Answer 22

All of them until proven otherwise

Question 23

2 benign kidney tumors?

Answer 23

1. Oncocytoma
2. Angiomyolipoma
   - Mimic malignant tumor
   * ***Usually removed surgically

Question 24

Characteristics of oncocytoma?

Answer 24

• Benign mahogany brown with central scar

- Usually removed surgically

Question 25

Characteristics of Angiomyolipoma?

Answer 25

- Consists of vessels, smooth muscles, fat - Vessels can rupture leading to hemorrhage - Can be associated with tubular sclerosis

Question 26

What is the triad of renal cell carcinoma?

Answer 26

"RCC" 1. Costovertebral pain 2. Hematuria 3. Large mass

Question 27

Which section of kidney cannot give rise to tumors?

Answer 27

Glomerulus

Question 28

Most common type of renal cancer?

Answer 28

1. Clear cell renal cell carcinoma 75% | 2. Papillary 15%

Question 29

Treatment of RCC?

Answer 29

- Surgery is mainstay | - Cancer is highly chemoresistant

Question 30

Survival rates with RCC stages?

Answer 30

Stage I: 96% Stage II: 82% Stage III: 64% Stage IV: 23%

Question 31

What is VHL?

Answer 31

"Von Hippel Lindau" - Familial RCC with early onset - Multiple, vascularized cysts and tumors - Have both renal cysts and tumors

Question 32

Most common pediatric renal cancer?

Answer 32

Wilms tumor

Question 33

Presentation of Wilms tumor?

Answer 33

75% have mass noticed by parents | 25% have other developmental abnormality

Question 34

Developmental disorders seen in Wilms tumor?

Answer 34

1. WAGR complex 2. Denys Drash syndrome 3. Beckwith - Wiedemann syndrome

Question 35

What is WAGR complex?

Answer 35

W - wilms tumor A - Aniridia G - GU malformation R - Mental retardation

Question 36

What is Denys Drash Syndrome?

Answer 36

`1. Wilms tumor 2. Gonadal dysgenesis 3. Renal sclerosis

Question 37

Treatment of Wilms tumor?

Answer 37

- Surgical excision - If it has spread, chemo - If not, not chemo but monitor closely

Question 38

Prime age for renal tumor?

Answer 38

65 yo

Question 39

65yo man with 5cm mass on right kidney?

Answer 39

- Schedule surgery - He is at prime age - Kidney masses are presumed malignant

Question 40

Which tumor associated with short arm chromosome III?

Answer 40

Clear cell

Question 41

Adult of pediatric renal tumors sensitive to chemo?

Answer 41

Pediatric

Question 42

What are immature cartilage and tubules characteristic of?

Answer 42

Cystic Dysplasia

Question 43

What are blastema, tubules, and stroma characteristic of?

Answer 43

Malignancy