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Question 1

What are oncocytomas?

Answer 1

benign tumors from the intercalated cells of the collecting ducts

Question 2

How common are oncocytomas?

Answer 2

represent less than 10+ of renal tumorsno clinical symptoms

Question 3

What chromosomal causes are associated with oncocytomas?

Answer 3

loss of chrom 1, 14, and Y

Question 4

T or F. Oncocytomas are more common in men

Answer 4

T.

Question 5

Description of oncocytomas

Answer 5

well-circumscribed tumor with a central scar

Question 6

What are the most common primary neoplasms in adults?

Answer 6

Clear cell carcinoma (70%)Papillary carcinoma (10%)Chromophobe carcinoma (5%)Oncocytoma (5%)Others (urothelial, squamous, 10%)

Question 7

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

Answer 7

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

Question 8

Usually the first sign of a kidney tumor is what?

Answer 8

painful hematuria (in elm’s it might actually be palpation of the mass)most kidney tumors have to get large before they cause symptoms

Question 9

Renal carcinomas tend to spread via what routes?

Answer 9

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

Question 10

What are the types of renal cell carcinoma?

Answer 10

-clear cell (most common)-papillary-chromophobe

Question 11

What are renal cell carcinomas derived from?

Answer 11

renal tubular epithelium located in cortex (polar)

Question 12

What patient population is common for renal cell carcinoma?

Answer 12

men in 60-70s

Question 13

What are the risk factors for renal cell carcinoma?

Answer 13

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

Question 14

Are clear cell carcinomas sporadic or familial?

Answer 14

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

Question 15

What does loss of VHL cause?

Answer 15

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

Question 16

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

Answer 16

Clinical stage…after that nuclear grade (Fuhrman)

Question 17

Why are renal cell carcinomas yellow grossly?

Answer 17

cells are filled with lipids

Question 18

How do papillary renal cell carcinomas present?

Answer 18

frequently multifocal and bilateral and appear as early-stage tumors

Question 19

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

Answer 19

size greater than 5 mm suggests papillary renal cell carcinoma

Question 20

Are papillary RCCs sporadic or hereditary?

Answer 20

can be either

Question 21

What are some potential causes of hereditary papillary RCC?

Answer 21

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

Question 22

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

Answer 22

a papillary fibrovascular core

Question 23

What do chromophobe carcinomas arise from?

Answer 23

Arise from intercalated cells of collecting ductsNo specific genetic signature, lots of different losses

Question 24

Prognosis for chromophobe carcinomas?

Answer 24

Good prognosis - Same behavior as clear cell when stratified by grade/stageTypical Fuhrman grading may NOT have prognostic significance

Question 25

Chromophobe carcinomas are positive for what?

Answer 25

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

Question 26

Chromophobe gross descriptor

Answer 26

No central scar

Question 27

What are the types of urothelial carcinomas?

Answer 27

-papillary (more granular and 3D)-flat (harder to diagnose)

Question 28

What is Wilm’s Tumor?

Answer 28

An embryonal pediatric tumor of the kidney

Question 29

When does Wilm’s Tumor present typically? Prognosis?

Answer 29

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%

Question 30

What causes Wilm’s Tumor?

Answer 30

Defects in WT1 gene chromosome 11There can be familial germline mutations (WAGR syndrome; Denys-Drash syndrome), but most Wilms tumors arise from acquired mutations

Question 31

Can you biopsy Wilm’s Tumor?

Answer 31

No, problems with seeding potential

Question 32

What else is Wilm’s tumor associated with?

Answer 32

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

Question 33

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

Answer 33

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

Question 34

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

Answer 34

-blastemal, -stromal, and -epithelial

Question 35

What are Angiomyolipoma’s associated with?

Answer 35

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