12.8 Renal Neoplasia

Question 1

Angiomyolipoma

Answer 1

Hamartoma comprised of blood vessels, smooth muscle and adipose tissue

Question 2

What condition are angiomyolipomas associated with?

Answer 2

Tuberous Sclerosis

Question 3

What is the cell of origin of renal cell carcinoma?

Answer 3

Epithelial cells of the renal tubule

Question 4

What is the triad of renal cell carcinoma?

Answer 4

• Hematuria
• Palpable mass
• Flank pain

Question 5

What is the most common presentation of renal cell carcinoma?

Answer 5

Hematuria

Question 6

What neoplastic syndromes may be associated with RCC?

Answer 6

• EPO release
• Renin release
• PTHrp release
• ACTH release

Question 7

What gene is often lost with RCC?

Answer 7

VHL (tumor suppressor gene)

Question 8

What is the key risk factor for sporadic RCC?

Answer 8

Smoking

Question 9

Are sporadic RCCs often unilateral or bilateral?

Answer 9

Unilateral

Question 10

Von Hippel-Lindau Disease

Answer 10

Autosomal dominant mutation that causes inactivation of VHL leading to increased risk fro hemangioblastoma of the cerebellum and RCC

Question 11

Wilms Tumor

Answer 11

Malignant renal tumor comprised of blastema, primitive glomeruli and tubules as well as stromal cells

Question 12

What is the most common renal tumor in children?

Answer 12

Wilms Tumor

Question 13

Beckwith-Wiedemann Syndrome

Answer 13

Associated with Wilms tumor and neonatal hypoglycemia. It is due to a mutation leading to increased expression of IGF-2.