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Pathoma - Kidney and Urinary Tract Pathology > Renal Neoplasia > Flashcards

Renal Neoplasia Flashcards

1
Q

What does Angiomyolipoma stand for?

A

Angio - blood vessel
Myo - muscle
Lipo - smooth muscle/fat

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2
Q

What is an Angiomyolipoma?

A

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

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3
Q

In what condition does Angiomyolipomas occur at an increased frequency?

A

Tuberous Sclerosis

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4
Q

What is Renal Cell Carcinoma?

A

Malignant epithelial tumor arising from kidney tubules.

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5
Q

What three symptoms are associated with Renal Cell Carcinoma?

A
  1. Hematuria
  2. Palpable Mass
  3. Flank Pain
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6
Q

What is the most common symptom of Renal Cell Carcinoma?

A

Hematuria

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7
Q

How often do all three symptoms associated with Renal Cell Carcinoma occur?

A

Rarely - Only 10% of patients

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8
Q

What three additional symptoms may also be present with Renal Cell Carcinoma?

A
  1. Fever
  2. Weight loss
  3. Paraneoplastic syndrome
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9
Q

What types of paraneoplastic syndromes can Renal Cell Carcinoma cause?

A
  1. Inc. EPO –> reactive polycythemia
  2. Inc. Renin –> HTN
  3. Inc. Parathyroid Hormon Related Peptied (PTHrP) –> hyercalcemia
  4. ACTH –> Cushing Syndrome
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10
Q

What may Renal Cell Carcinoma rarely present with in males?

A

Left-sided varicocele

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11
Q

What causes left sided variocele in Renal Cell Carcinoma?

A

Involvement of the left renal vein by carcinoma blocks drainage for the left spermatic vein leading to varicocele.

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12
Q

Why is right-sided varicocele usually not seen?

A

The right spermatic vein drains directly into the IVC.

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13
Q

What does Renal Cell Carcinoma look like upon gross exam?

A

Gross exam reveals a yellow mass

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14
Q

What is seen in Renal Cell Carcinoma microscopically?

A

The most common variant exhibits clear cytoplasm (clear cell type)

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15
Q

What is the genetic cause of Renal Cell Carcinoma?

A

Pathogenesis involves loss of VHL (3p) tumor suppressor gene

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16
Q

How does loss of VHL (3p) lead to tumor growth?

A

Loss of VHL (3p) –> Increased IGF-1 (promotes growth) and increased HIF transcription factor (increases VEGF and PDGF) –> these factors promote angiogenesis and growth

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17
Q

What are the two types of Renal Cell Carcinoma?

A

Tumors may be:
1. Hereditary
OR
2. Sporadic

18
Q

What do both types of Renal Cell Carcinoma involve?

A

Loss of the VHL gene

19
Q

What is the classic population that has Sporadic Renal Cell Carcinoma?

A

Classically arise in adult males (average age is 60 years)

20
Q

Where do Sporadic Renal Cell Carcinomas usually occur?

A

They occur as a single tumor in the upper pole of the kidney

21
Q

What is a major risk factor for sporadic renal cell carcinomas?

A

Cigarette smoke

22
Q

What is the classic population and presentation of Hereditary Renal Cell Carcinoma?

A

Bilateral tumors in young adults

23
Q

What is Von Hippel-Lindau disease associated with? How is it passed on?

A
  • Hereditary Renal Cell Carcinoma

- Autosomal dominant disorder

24
Q

What genetic cause is Von Hippel-Lindau disease associated with?

A

Associated with inactivation of the VHL gene

25
Q

What does inactivation of the VHL gene in Von Hippel-Lindau disease lead to?

A

Hemangioblastoma of the cerebellum & renal cell carcinoma

26
Q

What are two letters used for staging of Renal Cell Carcinoma?

A

T & N

27
Q

What does the T stage represent?

A

T - based on size and involvement of the renal vein

28
Q

How often does involvement of the renal vein with Renal Cell Carcinoma occur?

A

Occurs commonly.

29
Q

What does renal vein involvement with Renal Cell Carcinoma increase the risk of?

A

Hematogenous spread to the lungs and bone!

30
Q

What does the N stage represent?

A

N - Spread to retroperitoneal lymph nodes

31
Q

What is a Wilms Tumor and what is it composed of?

A

Malignant kidney tumor comprised of blastema (immature kidney mesenchyme), primitive glomeruli and tubules, and stromal cells

32
Q

What is blastema?

A
  • Primitive tissue from which the kidney forms!

- Immature kidney mesenchyme

33
Q

What is the most common malignant renal tumor in children? At what age does it occur on average?

A

Wilms Tumor, Age 3

34
Q

What does Wilms tumor usually present with?

A
  1. Large, unilateral flank mass
  2. Hematuria
  3. Hypertension (due to renin secretion)
35
Q

What percentage of cases of Wilms tumor are sporadic?

A

90%

36
Q

What three syndromes are syndromic wilms tumors seen in?

A
  1. WAGR syndrome
  2. Denys-Drash syndrome
  3. Beckwith-Wiedemann syndrome
37
Q

What is WAGR syndrome?

A

Wilms tumor
Aniridia (absence of the iris usually involving both eyes)
Genital abnormalities
Mental and Motor Retardation

38
Q

What genetic abnormality is WAGR syndrome associated with?

A

Deletion of WT1 tumor suppressor gene (located at 11p13)

39
Q

What three traits is Denys-Drash Syndrome associated with?

A
  1. Wilms tumor
  2. Progressive renal (glomerular) disease
  3. Male pseudohermaphroditism
40
Q

What genetic abnormality is Denys-Drash syndrome associated with?

A

Mutations in WT1

41
Q

What traits are associated with Beckwith-Wiedemann syndrome?

A
  • Wilms tumor
  • Neonatal hypoglycemia
  • Muscular hemihypertrophy
  • Organomegaly (including tongue)
42
Q

What genetic abnormality is associated with Beckwith-Wiedmann syndrome?

A

Mutations in WT2 gene cluster (imprinted genes at 11p15.5), particularly IGF-2

Pathoma - Kidney and Urinary Tract Pathology (9 decks)

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