10.30: Renal V Flashcards
What does the metanephric blastema develop into?
Nephrons
What does the ureteric bud develop into?
Collecting duct system
Are bilateral or unilateral lesions genetic?
Bilateral, unilateral cannot be genetic
What is agenesis?
Absence of something
What occurs in horseshoe kidneys?
They are fused
What is dysplasia?
Abnormal development
What is polycystic?
Cystic but without dysplastic elements
What is hypoplasia?
Small but no dysplasia demonstrated
What are enlarged irregular kidneys appearing like bunch of grapes characteristic of?
Cystic renal dysplasia
What is oligohydramnios?
- 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”
What is potters syndrome?
Hypoplastic lungs due to agenesis of kidneys
What is ARPKD?
Childhood “Autosomal recessive polycystic kidney disease”
- Enlarged kidneys with retained shape
- Liver cysts seen as well
What is APKD?
“Adult polycystic Kidney disease”
- More common than childhood recessive
- Autosomal dominant
- Huge kidney with cysts in liver as well
- Nephrons between cysts are functioning
Treatment of APKD?
- Transplant
What is often associated with APKD?
- Hepatic cysts
- Intracranial berry aneurysms
- Mitral prolapse
3 types of cystic disease?
- Developmental dysplasia
- APKD
- ARPKD
Only cystic disease without liver abnormalities?
Dysplasia
Only cystic disease with ureter abnormalities?
Dysplasia
Only cystic disease without reniform shape?
Dysplasia
What is acquired cystic disease associated with?
- Dialysis
- High rate of developing renal carcinoma
Which cystic disease has high risk of renal carcinoma?
Acquired
Which kidney tumors are presumed malignant?
All of them until proven otherwise
2 benign kidney tumors?
- Oncocytoma
- Angiomyolipoma
- Mimic malignant tumor
* ***Usually removed surgically
Characteristics of oncocytoma?
- Benign mahogany brown with central scar
- Usually removed surgically
Characteristics of Angiomyolipoma?
- Consists of vessels, smooth muscles, fat
- Vessels can rupture leading to hemorrhage
- Can be associated with tubular sclerosis
What is the triad of renal cell carcinoma?
“RCC”
- Costovertebral pain
- Hematuria
- Large mass
Which section of kidney cannot give rise to tumors?
Glomerulus
Most common type of renal cancer?
- Clear cell renal cell carcinoma 75%
2. Papillary 15%
Treatment of RCC?
- Surgery is mainstay
- Cancer is highly chemoresistant
Survival rates with RCC stages?
Stage I: 96%
Stage II: 82%
Stage III: 64%
Stage IV: 23%
What is VHL?
“Von Hippel Lindau”
- Familial RCC with early onset
- Multiple, vascularized cysts and tumors
- Have both renal cysts and tumors
Most common pediatric renal cancer?
Wilms tumor
Presentation of Wilms tumor?
75% have mass noticed by parents
25% have other developmental abnormality
Developmental disorders seen in Wilms tumor?
- WAGR complex
- Denys Drash syndrome
- Beckwith - Wiedemann syndrome
What is WAGR complex?
W - wilms tumor
A - Aniridia
G - GU malformation
R - Mental retardation
What is Denys Drash Syndrome?
`1. Wilms tumor
- Gonadal dysgenesis
- Renal sclerosis
Treatment of Wilms tumor?
- Surgical excision
- If it has spread, chemo
- If not, not chemo but monitor closely
Prime age for renal tumor?
65 yo
65yo man with 5cm mass on right kidney?
- Schedule surgery
- He is at prime age
- Kidney masses are presumed malignant
Which tumor associated with short arm chromosome III?
Clear cell
Adult of pediatric renal tumors sensitive to chemo?
Pediatric
What are immature cartilage and tubules characteristic of?
Cystic Dysplasia
What are blastema, tubules, and stroma characteristic of?
Malignancy
