Renal & Urinary Neoplasia

Question 1

What is an angiomyolipoma associated with?

Answer 1

Tuberous sclerosis

Question 2

How would you classify an angiomyolipoma?

Answer 2

Hamartoma

Question 3

Where does a renal cell carcinoma usually arise from?

Answer 3

Kidney tubules

Question 4

What is the classic triad of someone presenting with renal cell carcinoma?

Answer 4

Hematuria, palpable abdominal mass, flank pain –> rarely see all three together!

Question 5

Name some other symptoms associated with renal cell carcinoma?

Answer 5

1. Systemic - fever, weight loss
2. Paraneoplastic syndromes: reactive polycythemia, HTN, Hypercalcemia, Cushing’s
3. Left-sided varicocele (rare)

Question 6

What does renal cell carcinoma kidneys look like

1. Gross appearance
2. Microscopic appearance

Answer 6

1. Yellow mass

2. Clear cytoplasm (most common)

Question 7

Which gene is affected in renal cell carcinoma? What chromosome is it on?

Answer 7

Tumor suppressor gene VHL on chromosome 3p

Question 8

What are the consequences of the loss of the gene involved in renal cell carcinoma?

Answer 8

1. Increased IGF-1 –> promotes growth

2. Increased HIF –> promotes VEGF & PDGF

Question 9

Sporadic renal cell carcinoma tumors are commonly seen in which population group? Where is this tumor classically located?

Answer 9

Adult males (peak in 60s); single tumor in upper pole of kidney

Question 10

What is the greatest risk factor for developing a sporadic renal cell carcinoma?

Answer 10

Cigarette smoke!!!

Question 11

Hereditary renal cell carcinoma tumors are commonly seen in which population group? Where is this tumor classically located?

Answer 11

= Von Hippel Lindau disease; in younger adults; often bilateral tumors

Question 12

Besides renal cell carcinoma, what else is Von Hippel Lindau disease associated with?

Answer 12

Most commonly hemangioblastomas in cerebellum; but tumors can also form in KAPICKE: kidney 1, adrenal gland, pancreas, inner ear, CNS, kidney 2, eyes

Question 13

What is the inheritance pattern for von Hippel lindau disease?

Answer 13

Autosomal dominant

Question 14

What vein is commonly involved in renal cell carcinoma? What risk does this impose?

Answer 14

Renal vein –> can allow hematogenous spread to lungs and bones

Question 15

What lymph nodes does renal cell carcinoma typically spread to?

Answer 15

Retroperitoneal lymph nodes

Question 16

A Wilms tumor is composed of which types of cells?

Answer 16

Blastema = immature kidney mesenchyme
Primitive glomeruli and tubules
Stromal cells

Question 17

What is the most common malignant renal tumor in children?

Answer 17

Wilms tumor

Question 18

What is the typical presentation of someone with a Wilms tumor?

Answer 18

Three year old with a large, unilateral flank mass, hematuria, HTN

Question 19

What mutation is a Wilms tumor associated with?

Answer 19

WWTI mutation

Question 20

What are the two types of syndromes a Wilms tumor can result in?

Answer 20

1. WAGR Syndrome: Wilms tumor, Aniridia, Genital abnormalities, mental/motor Retardation
2. Beckwith-Wiedermann Syndrome: Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, organomegaly

Question 21

What are the three types of lower urinary tract carcinomas?

Answer 21

1. Transitional cell –> urothelial carcinoma (entire LUT, usually bladder)
2. Squamous cell carcinoma (usually bladder)
3. Glandular cells –> adenocarcinoma (usually bladder)

Question 22

What is the most common type of lower urinary tract carcinoma?

Answer 22

Urothelilal/ transitional cell carcinoma

Question 23

What region do lower urinary tract cancers usually involve?

Answer 23

Bladder

Question 24

What is the major risk factor for urothelial carcinoma? Can you name some others?

Answer 24

Cigarette smoke! Others include napthylamine, azo dyes, long-term cyclophosphamide or phenacetin use

Question 25

Describe the classic clinical presentation of someone with urothelial carcinoma.

Answer 25

Older adult presenting with PAINLESS hematuria

Question 26

What are the two pathways through which urothelial carcinomas can develop?

Answer 26

1. Flat | 2. Papillary

Question 27

Describe features of urothelial carcinoma developing by the flat pathway.

Answer 27

1. Starts as high-grade flat tumor then invades | 2. Associated with early p53 mutations

Question 28

Describe features of urothelial carcinoma developing by the papillary pathway.

Answer 28

1. Starts as low-grade papillary then progresses to high-grade papillary and then invades 2. Not associated with early p53 mutations

Question 29

Describe the 'field defect' associated with urothelial carcinomas.

Answer 29

Entire urothelial surface hit with carcinogens --> entire field mutated --> see multifocal tumors that recur

Question 30

What usually precedes a squamous cell carcinoma?

Answer 30

Squamous metaplasia --> normal bladder surface is usually transitional epithelium: Squamous metaplasia --> dysplasia --> carcinoma

Question 31

Name three risk factors for developing squamous cell carcinoma.

Answer 31

1. Chronic cystitis (older woman) 2. Schistosoma hematobium infection (Egyptian male) 3. Long-standing nephrolithiasis (chronic irritation leads to development of metaplasia)

Question 32

What are three conditions from which an adenocarcinoma of the bladder arises?

Answer 32

1. Urachal remnant --> urachus lined by grandular cells & passes through dome of bladder 2. Cystitis glandularis: chronic bladder inflammation --> columnar metaplasia 3. Exstrophy --> congenital failure to form caudal portion of anterior abdominal & bladder walls --> bladder surface exposed to outside world