Renal Neoplasia - Pathoma Flashcards

1
Q

What is an Angiomyolipoma?

A

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

  • Angio = blood vessel
  • myo = muscle
  • lipo = fat (adipose tissue)
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2
Q

In what cases is there an increased frequency of Angiomyolipomas?

A

Tuberous sclerosis

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3
Q

What type of Renal neoplasia is a malignant epithelial tumor that arises from the kidney tubules?

A

Renal Cell Carcinoma

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4
Q

What is the classic presentation of Renal Cell Carcinoma?

A

Triad:

  • Hematuria
  • Palpable mass
  • Flank pain Also common:
  • fever
  • weight loss
  • paraneoplastic syndrome (EPO, renin, parathyroid horomone related peptide, ACTH)
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5
Q

How does a varicocele form in the case of Renal Cell Carcinoma?

A

Left-side only

  • carcinoma obstructs gonadal vein draining into left renal vein
  • blood backs up => varicocele Not on right side
  • gonadal veing drains directly into inferior vena cava
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6
Q

Which type of renal neoplasm reveals a yellow mass on gross exam and clear cytoplasm microscopically?

A

Renal Cell Carcinoma(called clear cell type)

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7
Q

The pathogenesis of Renal Cell Carcinoma involves the loss of what gene?

A

VHL (3p) = tumor suppressor gene

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8
Q

What happens when you lose the expression of VHL in Renal Cell Carcinoma?

A

Increased IGF-3 (insulin-like growth factor)

  • promotes growth Increased HIF transcription factor
  • increases VEGF and PDGF (platelet-derived growth factor)
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9
Q

What are the two pathways in the development of Renal Cell Carcinoma?

A
Sporadic Pathway			
* single tumor in upper pole of kidney		
* usually in smoker			Hereditary			
* tumors that arise in younger patients		
* multiple tumors and bilateral		
***Both involve loss of VHL expression!
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10
Q

What is one autosomal dominant hereditary disorder that is associated with the inactivation of the VHL gene?

A

Von-Hippel Lindau Disease

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11
Q

Patients with Von Hippel-Lindau are at increased risk to develop what?

A
  • Hemangioblastoma of the cerebellum

* Renal Cell Carcinoma

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12
Q

What is staging of Renal Cell Carcinoma based on?

A

T = based on tumor size and involvement of renal vein

  • increases the risk of hematogenous spread to the lungs and bones
  • N = spread to retroperitoneal lymph nodes
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13
Q

What do you call a malignant tumor of the kidney comprised of blastema, primitive glomeruli and tubules, and stromal cells?

A

Wilms Tumor

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14
Q

What is the most common malignant renal tumor in children?

A

Wilms Tumor(average age = 3 yoa)

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15
Q

What is the classic presentation of Wilms Tumor?

A
  • Large, unilateral flank mass
  • Hematuria HTN
  • due to renin secretion
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16
Q

What mutation is Wilms Tumor associated with?

A

WT1 mutations(especially syndromic cases)

17
Q

What is the WAGR Syndrome?

A
  • W = Wilm’s tumor
  • A = Aniridia
  • G = Genital abnormalities
  • R = Mental and motor Retardation
18
Q

What WT1 mutation associated syndrome consists of Wilms Tumor, neonatal hypoglycemia, muscular hemihypertrophy, and organomegaly (e.g. tongue)?

A

Beckwith-Wiedmann Syndrome