Renal Neoplasms

Question 1

Most common primary tumor of the kidney in children.

Answer 1

Wilm’s Tumor/ Nephroblastoma

Question 2

Wilm’s tumor/Nephroblastoma is seen in kids what ages?

Answer 2

2-5 Toddlers

Question 3

What are the 2 ways you can get Wilm’s tumor?

Answer 3

Sporadic - 90% of cases

Syndromic - associated with 3 syndromes..

Question 4

Name the 3 syndromes associated with development of Wilm’s Tumor.

Answer 4

WAGR

Denys-Drash

Beckwith-Wiedemann

Question 5

Abnormalities of what chromosome are associated with Wilm’s tumor in children?

Answer 5

Chromosome 11 - WT1 gene and WT2-gene cluster

specific WT2 gene has not been ID’d

Question 6

Deletion of WT1 gene is associated with what?

Answer 6

WAGR syndrome

Question 7

Mutations of WT1 gene….

Answer 7

Denys-Drash syndrome

Question 8

Which syndrome is associated with increased size of body organs, especially tongue? What genetic abnormality causes Wilm’s tumor in this syndrome?

Answer 8

Beckwith-Wiedemann - Genomic imprinting disorder.

Overexpression of IGF-2 (insulin like growth factor 2) protein, resulting in organ enlargement and tumorigenesis.

Question 9

What tumor is associated with loss of Chromosomes 1, 14, and Y chromosomes?

Answer 9

Oncocytomas

Question 10

Are Oncocytomas benign or malignant?

Answer 10

Benign

Question 11

what is the characteristic histology of an oncocytoma?

Answer 11

MITOCHONDRIA EVERYWHERE

Question 12

On autopsy, what is the characteristic gross feature of oncocytomas?

Answer 12

Round, with a central stellate scar

Question 13

Name the most common malignant renal neoplasm.

Answer 13

Renal cell carcinoma, specifically Clear Cell Carcinoma

Question 14

Why are the cells clear?

Answer 14

Vacuoles filled with fat. Yellow color on gross observation.

Question 15

Clear cell Carcinomas have what genetic abnormality?

Answer 15

2-hit mutations in the VHL tumor suppressor gene. Leads to uninhibited HIF (hypoxia inducible factor) activation of VEGF transcription.

Question 16

What is the first symptom of Clear Cell Carcinoma? Why?

Answer 16

Painless Hematuria –> tons of weak, new vessels due to VEGF running rampant.

Question 17

What’s the generic patient you’ll see with renal cell carcinomas? (specifically… what predisposes you to it..)

Answer 17

65 year old male SMOKER

Question 18

How do Renal cell Carcinomas spread?

Answer 18

They spread equally between lymph and hematogenous. They like to invade the RENAL VEIN.

Question 19

What are the 2 most common sites of metastasis of Renal Cell Carcinomas?

Answer 19

Lung - veins flow to lung

Bones (scapula) - nearest thing to it…

Question 20

What is the second most common type of Renal Cell Carcinoma? How do you differentiate it from Clear Cell?

Answer 20

Papillary Renal Cell carcinoma.

Tend to be MULTIFOCAL and BILATERAL. Histology won’t show clear cells.

Question 21

What mutation is associated with Papillary Renal Cell Carconimas?

Answer 21

MET proto-oncogene activating mutations.

MET is a tyrosine kinase receptor that’s activated by HGF (Hepatocyte growth factor)

“Have you MET PAP?”

Question 22

What is the least common subset of Renal cell carcinoma?

Answer 22

Chromophobe Renal cell carcinoma.

Question 23

What genetic abnormality is associated with Chromophobe Renal Cell Carconimas?

Answer 23

Total loss of multiple chromosomes. HYPODIPLOIDY.

Question 24

What is the prognosis for each of the subsets of Renal Cell Carcinoma?

Answer 24

1. Clear Cell - Bad
2. Papillary - Bad
3. Chromophobe - Good

Question 25

What cell population does a Chromophobe Renal Cell Carcinoma arise from?

Answer 25

The UROTHELIUM of the RENAL PELVIS. (transitional epithelium)

Question 26

What population of cells does Papillary Renal Cell Carcinoma and Clear Cell ….arise from?

Answer 26

Tubular epithelial cells