Cancer

Question 1

Angiomyolipoma

Answer 1

• most common benign renal tumor made up of blood vasculature, smooth muscle, and fat
• associated with tuberous sclerosis

Question 2

The most common benign renal tumor is ____________; most common malignancy is ____________.

Answer 2

• benign: angiomyolipoma

- malignant: renal cell carcinoma (RCC)

Question 3

Renal Cell Carcinoma

Answer 3

• most common renal malignancy; arises from the kidney tubules (epithelium)

Question 4

Paraneoplastic Syndromes of RCC

Answer 4

• excess EPO, renin (HTN), PTHrP (hypercalcemia), ACTH (Cushing’s)

Question 5

What can occur in RCC of the left kidney? Why?

Answer 5

• left-sided varicocele (swollen scrotum)
• RCC commonly invades the renal veins; the left testicular vein dumps into the left renal vein, so if the left renal vein gets obstructed, the resulting back flow can clog up the left testicular vein = swelling

Question 6

Gross examination of RCC shows a:

Answer 6

• large yellow mass

Question 7

What is the most common sub-type of RCC? Where does it get its name from?

Answer 7

• clear cell RCC

- it is called this because these cells have a clear cytoplasm

Question 8

T or F: most RCC is hereditary.

Answer 8

• false!
• most are sporadic
• sporadic usually presents with a unilateral, single tumor
• hereditary/familial usually presents with bilateral, multifocal tumors

Question 9

What are the three most common types of RCC?

Answer 9

• clear cell RCC (majority of cases)
• papillary RCC (~15%)
• chromophobe RCC (3rd most common)

Question 10

Which type of RCC is the most common in patients with Renal Cystic Disease?

Answer 10

• papillary RCC

Question 11

The loss of which TSG is associated with RCC? Which chromosome is it located on?

Answer 11

• loss of the VHL tumor-suppressor-gene

- it is found on chromosome 3p

Question 12

Von Hippel-Lindau Disease

Answer 12

• an autosomal dominant loss of the VHL tumor-suppressor-gene on chromosome 3p
• pre-disposes patients to RCC and hemangioblastoma of the cerebellum

Question 13

Why lymph nodes to RCCs tend to invade?

Answer 13

• the retroperitoneal lymph nodes

Question 14

Wilms Tumor / Nephroblastoma

Answer 14

• the most common renal malignancy in children
• large unilateral flank mass
• triphasic: blastemal, stromal, and epithelial components

Question 15

What should you consider in a child with hematuria and HTN?

Answer 15

• renal malignancy; specifically, Wilms tumor (nephroblastoma)

Question 16

What are the three most common malignancies of the lower urinary tract?

Answer 16

• urothelial carcinoma (most common), squamous cell carcinoma, and adenocarcinoma

Question 17

Urothelial Carcinoma is a malignancy arising from the ___________; therefore, which parts of the lower urinary tract can it develop in?

Answer 17

• a malignancy of the urothelial lining
• because this lining is continuous throughout the entire tract, urothelial carcinoma can develop in the renal pelvis, the ureter, the bladder, or the urethra

Question 18

Each lower urinary tract carcinoma most commonly affects what part of the tract?

Answer 18

• the bladder

Question 19

Why are tumors in Urothelial Carcinoma usually multi-focal and highly recurrent?

Answer 19

• because the urothelial lining is continuous, so once it becomes malignant it can easily spread and re-appear throughout the tract

Question 20

What are the two pathways of Urothelial Carcinoma? Which one is associated with early p53 mutations?

Answer 20

• flat pathway: flat lesion of tumor cells forms (starts as a high-grade tumor)
• papillary pathway: tumors develop as a papillary growth (start as a low-grade tumor and progress to high-grade)
• the flat pathway is associated with early p53 mutations

Question 21

Why is it surprising to find Squamous Cell Carcinoma in the lower urinary tract? (ie: What does it require?)

Answer 21

• it’s strange because squamous cells are not found in the urinary tract
• therefore, squamous cell carcinoma requires previous squamous metaplasia of the tract

Question 22

What are common risk factors for squamous metaplasia of the lower urinary tract (and therefore for Squamous Cell Carcinoma)?

Answer 22

• chronic cystitis
• chronic inflammation (esp. via Schistasoma hematabium in the Middle East)
• long-standing nephrolithiasis (kidney stone)

Question 23

Why is it surprising to find Adenocarcinoma in the lower urinary tract?

Answer 23

• glandular tissue is not normally present in the lower urinary tract

Question 24

How can glandular tissue be present in the lower urinary tract (3 particular examples)?

Answer 24

• urachal remnant
• cystitis glandularia
• bladder exstrophy

Question 25

Urachal Remnant

Answer 25

- a unique circumstance causing glandular tissue to be in the urinary tract (so it can lead to adenocarcinoma) - a remnant of the urachus, which is the duct connecting the fetal bladder to the umbilical cord; it is lined with glandular cells

Question 26

Cystitis Glandularia

Answer 26

- a unique circumstance causing glandular tissue to be in the urinary tract (so it can lead to adenocarcinoma) - columnar metaplasia that can progress to glandular metaplasia due to chronic inflammation of the bladder

Question 27

Bladder Exstrophy

Answer 27

- a unique circumstance causing glandular tissue to be in the urinary tract (so it can lead to adenocarcinoma) - a congenital failure to form the anterior portion of the abdominal wall, resulting in the protrusion of the urinary bladder - this exposure to the outside can result in glandular metaplasia

Question 28

_______-_______ Syndrome is associated with Wilms Tumor. What does it entail?

Answer 28

- Beckwith-Weidemann Syndrome | - Wilms tumor, neonatal hypoglycemia, muscular hemi-hypertrophy, organomegaly

Question 29

_._._._. Syndrome is associated with Wilms Tumor. What does it entail?

Answer 29

- W.A.G.R. Syndrome - Wilms tumor - Aniridia (no irises) - Genital abnormalities - Retardation (mental)

Question 30

What is the classic triad of symptoms in RCC?

Answer 30

- hematuria, palpable mass, and flank pain | - (all are seen in 10% of cases)