Pathoma Renal Neoplasia

Question 1

Angiomyolipoma and where we see it most

Answer 1

Hamartoma comprised of blood vessels, smooth muscle, and adipose tissue

Increased frequency in tuberous sclerosis

Question 2

Presentation of renal cell carcinoma

Answer 2

Malignant, presents with triad

1. hematuria
2. palpable mass
3. flank pain

Question 3

Systemic problem with renal cell carcinoma

Answer 3

Many paraneoplastic syndromes

EPO (Polycythenia), renin (cause HTN), PTHrP (hypercalcemia) or ACTH (Cushings) can be released

Question 4

Rarely, a renal cell carcinoma can present with this anatomical abnormality

Answer 4

Left sided varicocele, which happens because he bloackage at the renal vein also blocks the spermatic vein trying to drain into the IVC (that’s why it is only on the left, because the Right drains directly into the IVC)

Question 5

Gross exam of renal cell carcinoma

Answer 5

Yellow mass with most commonly a clear cytoplasm (clear cell type)

Question 6

Genetic cause of renal cell carcinoma

Answer 6

Loss of VHL (3p) tumor suppressor gene.

This causes an increased IGF-1 which promotes growth and an increased HIF transcription factor which increases VEGF and PDGF

Question 7

Sporadic vs. hereditary renal cell carcinoma

Answer 7

Sporadic - Adult smoker, one mass at the upper pole

Hered - Younger patients, bilateral, many tumors

Both involve loss of VHL

Question 8

Von Hippel-Lindau Disease

Answer 8

Autosomal dominant disorder associated with inactivation of VHL

Increased risk for hemangioblastoma of the cerebellum and renal cell carcinoma

Question 9

T vs. N stage renal cell carcinoma

Answer 9

T - going to renal vei nand spreading

N - Lymph nodes

Question 10

Wilms Tumor and who gets it

Answer 10

Malignant kidney tumor comprised of blastema (primitive tissue kidney is made of), primitive glomeruli and tubules and stromal cells

Most common malignant renal tumor in children. Average age of 3 years

Question 11

Wilms Tumor presentation

Answer 11

Large unilateral flank mass with hematuria and HTN from renin secretion

Question 12

Mutation with Wilms tumor

Answer 12

WT1 mutation

Question 13

Association with Wilms Tumor

Answer 13

Syndromic cases

Beckwith-Wiedmann syndrome

Question 14

Most high yield syndromic case?

Answer 14

WAGR syndrome

Wilms tumor
Aniridia
Genital abnormalities
mental Retardation

Question 15

Beckwith Wiedmann syndrome

Answer 15

WONM

Wilms
Neonatal hypoglycemia
Muscular hemihypertrophy
Organomegaly