12.8 Renal Neoplasia

Question 1

paraneoplastic syndrome

• what is it
• examples (4)

Answer 1

tumors that excrete hormones

1. EPO
2. Renin
3. PTHrP
4. ACTH

Question 1

large, unilateral flank mass, hematuria, HTN

-think what

Answer 1

Wilm’s tumor

-HTN due to renin secretion

Question 2

renal cell carcinoma:

-classic symptoms

Answer 2

Classic triad:

1. hematuria
2. flank pain
3. palpable mass

however, only 10% does triad of symptoms actually happen

Question 3

Wilms tumor

-classic symptoms

Answer 3

1. large, unilateral palpable mass
2. hematuria
3. HTN (from renin secretion)

Question 4

hematuria, palpable mass, flank pain

-think what

Answer 4

think RCC

Question 4

Denys-Drash syndrome

what is it (3 things)

how related to kidney

assoc with what pathogenesis

Answer 4

1. Wilms tumor,
2. progressive glomerular disease
3. male pseudohermaphroditism

assoc with WT1 mutations

Question 5

RCC: what you see on:

gross exam

histology

Answer 5

1. yellow mass
2. clear cytoplasm (‘clear cell type’) is common

Question 6

renal tumor in a child, think what?

Answer 6

Wilm’s tumor

Question 7

Beckwith-Wiedemann syndrome

symptoms (4)

Answer 7

1. Wilms tumor
2. neonatal hypoglycemia
3. muscular hemihypertrophy
4. organomegaly (including tongue)

WT2 mutations

Question 8

renal cell carcinoma

-in addition to classic symptoms, what additional ones to know? (4)

Answer 8

classic triad: palpable mass, flank pain, hematuria

1. fever
2. weight loss
3. paraneoplastic syndrome! (eg EPO, renin, PTHrP, ACTH)
4. left sided varicocele (remember anatomy)

Question 8

RCC:

how do hereditary and sporadic types present differently?

Answer 8

1. age: sporadic is adult males >60, hereditary is younger adults
2. sporadic: usu unilateral, hereditary: often bilateral

Question 9

WAGR syndrome

• what is it
• how related to kidney
• associated with what pathogenesis

Answer 9

• Wilms tumor
• Aniridia–no iris
• Genital abnormalities
• Retardation (mental and motor)
• assoc with deletion of WT1 tumor suppressor gene

Question 10

why would varicocele present with RCC?

which side?

Answer 10

left side b/c spermatic vein drains into L renal vein

(RCC loves to invade renal vein)

Question 12

Wilm’s tumor

-classic population

Answer 12

-most common malignant renal tumor in children

Question 14

angiomyolipoma

• what is it
• what is the important association?

Answer 14

• all in the name: hamartoma of blood vessels, muscles, and adipose tissue
• assoc with tuberous sclerosis

Question 15

RCC:

describe pathogenesis

Answer 15

• loss of VHL (3p) tumor suppressor gene, leads to:
  1. increased IGF-1 (growth factor)
  2. increased HIF (hypoxia induceable factor) transcription factor (increases VEGF and PDGF)

Question 17

RCC:

describe types

Answer 17

1. sporadic
   - classically in adult males >60, smoking is major risk factor, classically single tumor in upper pole of kidney
2. hereditary
   - younger adults, often bilateral
   - Von Hippel Lindau disease is associated with VHL gene inactivation, increasing risk for RCC

Question 18

Wilm’s tumor

-what syndromes may you see it with? (3)

Answer 18

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

Question 19

Wilm’s tumor

-made of what, mech

Answer 19

-made of blastema (immature kidney mesenchyme), primitive glomeruli and tubules, and stromal cells

Question 20

Von Hippel Lindau disease

• how assoc with kidney?
• what else increased risk?

Answer 20

• increased risk of hereditary RCC
• b/c VHL disease is inactivation of VHL gene.
• also increased risk for hemangioblastoma of cerebellum