Renal & Urinary Neoplasia Flashcards

1
Q

What is an angiomyolipoma associated with?

A

Tuberous sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

How would you classify an angiomyolipoma?

A

Hamartoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Where does a renal cell carcinoma usually arise from?

A

Kidney tubules

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Name some other symptoms associated with renal cell carcinoma?

A
  1. Systemic - fever, weight loss
  2. Paraneoplastic syndromes: reactive polycythemia, HTN, Hypercalcemia, Cushing’s
  3. Left-sided varicocele (rare)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What does renal cell carcinoma kidneys look like

  1. Gross appearance
  2. Microscopic appearance
A
  1. Yellow mass

2. Clear cytoplasm (most common)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

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

A

Tumor suppressor gene VHL on chromosome 3p

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

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

A
  1. Increased IGF-1 –> promotes growth

2. Increased HIF –> promotes VEGF & PDGF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

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

A

Cigarette smoke!!!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the inheritance pattern for von Hippel lindau disease?

A

Autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What lymph nodes does renal cell carcinoma typically spread to?

A

Retroperitoneal lymph nodes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

A Wilms tumor is composed of which types of cells?

A

Blastema = immature kidney mesenchyme
Primitive glomeruli and tubules
Stromal cells

17
Q

What is the most common malignant renal tumor in children?

A

Wilms tumor

18
Q

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

A

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

19
Q

What mutation is a Wilms tumor associated with?

A

WWTI mutation

20
Q

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

A
  1. WAGR Syndrome: Wilms tumor, Aniridia, Genital abnormalities, mental/motor Retardation
  2. Beckwith-Wiedermann Syndrome: Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, organomegaly
21
Q

What are the three types of lower urinary tract carcinomas?

A
  1. Transitional cell –> urothelial carcinoma (entire LUT, usually bladder)
  2. Squamous cell carcinoma (usually bladder)
  3. Glandular cells –> adenocarcinoma (usually bladder)
22
Q

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

A

Urothelilal/ transitional cell carcinoma

23
Q

What region do lower urinary tract cancers usually involve?

A

Bladder

24
Q

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

A

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

25
Q

Describe the classic clinical presentation of someone with urothelial carcinoma.

A

Older adult presenting with PAINLESS hematuria

26
Q

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

A
  1. Flat

2. Papillary

27
Q

Describe features of urothelial carcinoma developing by the flat pathway.

A
  1. Starts as high-grade flat tumor then invades

2. Associated with early p53 mutations

28
Q

Describe features of urothelial carcinoma developing by the papillary pathway.

A
  1. Starts as low-grade papillary then progresses to high-grade papillary and then invades
  2. Not associated with early p53 mutations
29
Q

Describe the ‘field defect’ associated with urothelial carcinomas.

A

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

30
Q

What usually precedes a squamous cell carcinoma?

A

Squamous metaplasia –> normal bladder surface is usually transitional epithelium:

Squamous metaplasia –> dysplasia –> carcinoma

31
Q

Name three risk factors for developing squamous cell carcinoma.

A
  1. Chronic cystitis (older woman)
  2. Schistosoma hematobium infection (Egyptian male)
  3. Long-standing nephrolithiasis (chronic irritation leads to development of metaplasia)
32
Q

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

A
  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