Renal Neoplasia Flashcards

1
Q

What are oncocytomas?

A

benign tumors from the intercalated cells of the collecting ducts

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2
Q

How common are oncocytomas?

A

represent less than 10+ of renal tumors

no clinical symptoms

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3
Q

What chromosomal causes are associated with oncocytomas?

A

loss of chrom 1, 14, and Y

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4
Q

T or F. Oncocytomas are more common in men

A

T.

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5
Q

Description of oncocytomas

A

well-circumscribed tumor with a central scar

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6
Q

What are the most common primary neoplasms in adults?

A

Clear cell carcinoma (70%)

Papillary carcinoma (10%)

Chromophobe carcinoma (5%)

Oncocytoma (5%)

Others (urothelial, squamous, 10%)

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7
Q

One thing that can help differentiate kidney tumors is location. Where do urothelial and squamous tumors tend to be?

Test question

A

near the renal pelvis while the others are at the poles of the kidney

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8
Q

Usually the first sign of a kidney tumor is what?

A

painful hematuria (in elm’s it might actually be palpation of the mass)

most kidney tumors have to get large before they cause symptoms

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9
Q

Renal carcinomas tend to spread via what routes?

A

either through Gerota’s fascia or into the renal vein and up through the IVC

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10
Q

What are the types of renal cell carcinoma?

A
  • clear cell (most common)
  • papillary
  • chromophobe
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11
Q

What are renal cell carcinomas derived from?

A

renal tubular epithelium

located in cortex (polar)

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12
Q

What patient population is common for renal cell carcinoma?

A

men in 60-70s

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13
Q

What are the risk factors for renal cell carcinoma?

A
  • *smokers (same for bladder cancer),
  • hypertensive or obese patients,
  • occupational exposure to cadmium,
  • acquired polycystic disease in chronic dialysis patients
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14
Q

Are clear cell carcinomas sporadic or familial?

A

Most are sporadic, however familial forms do exist as those with loss of VHL (autosomal dominant, chromosome 3)

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15
Q

What does loss of VHL cause?

A

Without VHL, HIFs are stabilized. HIFs are transcription factors that contribute to carcinogenesis by stimulating the expression of vascular endothelial growth factor (VEGF), an important angiogenic factor

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16
Q

What is the most important prognostic feature of renal cell carcinoma?

A

Clinical stage…after that nuclear grade (Fuhrman)

17
Q

Why are renal cell carcinomas yellow grossly?

A

cells are filled with lipids

18
Q

How do papillary renal cell carcinomas present?

A

frequently multifocal and bilateral and appear as early-stage tumors

19
Q

How can papillary renal cell carcinomas be distinguished from papillary adenomas?

A

size greater than 5 mm suggests papillary renal cell carcinoma

20
Q

Are papillary RCCs sporadic or hereditary?

A

can be either

21
Q

What are some potential causes of hereditary papillary RCC?

A

Chromosome 7 alterations that increase dosage of the MET proto-oncogene seems to spur abnormal growth in the proximal tubular epithelial cell precursors of papillary carcinomas

22
Q

What has to be present to be a true papillary lesion?

A

a papillary fibrovascular core

23
Q

What do chromophobe carcinomas arise from?

A

Arise from intercalated cells of collecting ducts

No specific genetic signature, lots of different losses

24
Q

Prognosis for chromophobe carcinomas?

A

Good prognosis - Same behavior as clear cell when stratified by grade/stage

Typical Fuhrman grading may NOT have prognostic significance

25
Q

Chromophobe carcinomas are positive for what?

A

Positive for Hale’s colloidal iron stain (negative in oncocytomas)

26
Q

Chromophobe gross descriptor

A

No central scar

27
Q

What are the types of urothelial carcinomas?

A
  • papillary (more granular and 3D)

- flat (harder to diagnose)

28
Q

What is Wilm’s Tumor?

A

An embryonal pediatric tumor of the kidney

29
Q

When does Wilm’s Tumor present typically? Prognosis?

A

The peak incidence of Wilms tumor is between the second and fifth year of life (95% of kidney cancer in children).

Post therapy 5 yr survival 90%

30
Q

What causes Wilm’s Tumor?

A

Defects in WT1 gene chromosome 11

There can be familial germline mutations (WAGR syndrome; Denys-Drash syndrome), but most Wilms tumors arise from acquired mutations

31
Q

Can you biopsy Wilm’s Tumor?

A

No, problems with seeding potential

32
Q

What else is Wilm’s tumor associated with?

A

Beckwith Wiedemann syndrome- one side of the body is a little larger than the other

33
Q

The presence of anaplasia with Wilm’s tumor correlates with what?

A

The presence of acquired TP53 mutations, and the emergence of resistance to chemotherapy.

34
Q

Microscopically Wilm’s tumors composed of a mixture of what cellular elements?

A
  • blastemal,
  • stromal, and
  • epithelial
35
Q

What are Angiomyolipoma’s associated with?

A

Patients with Tuberous Sclerosis (TSC1/2 mutation encoding hamerin and tuberin)