Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pathophysiology - Urinary Tract > Tumors of Urinary Tract > Flashcards

Tumors of Urinary Tract Flashcards

1
Q

What is a renal papillary adenoma / where is it located and how does it look under the microscope?

A

Benign tumor of renal tubular epithelium invariably located in the renal cortex (also called renal cortical adenoma)

Appears as cytologically bland epithelial cells composed of branching papillary structures

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

How do renal cortical adenomas present clinically, and what is the diagnosis restricted to in terms of size?

A

Typically an incidental finding on kidney resection for other findings or autopsy

Low grade lesion which must be 5mm or less in size

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

What cells are an oncocytoma comprised of? Is it benign or malignant?

A

Comprised of oncocytes - renal collecting duct intercalated epithelial cells which stain very eosinophilically due to abundant mitochondria and lack of perinuclear clearing

It is a benign tumor

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

How does oncocytoma appear grossly? What will be the patient’s presenting symptoms?

A

Usually asympatomatic, but may have hematuria / flank pain

Appear as a sometimes-large, well circumscribed brown mass with a central white scar

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

What is the most common primary malignancy of the kidney? What is the cell of origin? How old / what sex are patients who generally present with it?

A

Renal cell carcinoma (RCC)

Cell of origin is proximal renal tubular epithelial cells

Patients are generally older males (i.e. 60)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the three most common types of RCC? Which have the best prognosis?

A
  1. Clear cell RCC
  2. Papillary RCC
  3. Chromophobe RCC

Papillary / chromophobe better prognosis than clear cell

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

What are the major risk factors for sporadic RCCs?

A

Cigarette smoking and obesity

Long-term dialysis with acquired cystic kidney disease

Hypertension

Occupational Exposures - cadmium, organic solvents (trichloroethylene in plastics), drugs like cyclophosphamide and phenacetin

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

What hereditary condition is most commonly associated with development of RCC and what chromosome is the gene found on?

A

Von Hippel Lindau (3 letters)

Autosomal dominant - tumor suppression gene on chromosome 3 (3 letters = RCC) called VHL

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

What is the function of VHL / how do things go awry?

A

Tumor suppressor gene which inhibits hypoxia inducible factor
-> defect leads to increased transcription of HIF -> increased transcription of VEGF and PDGF

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

What are the most common benign / malignant tumors associated with von Hippel Lindau?

A

Hemangioblastomas - especially cerebellum
Pheochromocytomas
Bilateral renal cell carcinomas, happening at younger ages

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

What inherited genetic disorder is associated with development of papillary RCC? What gene product has gone awry?

A

hereditary papillary renal cell carcinoma (HPRCC)

Autosomal dominant, due to mutation in a tyrosine kinase of MET proto-oncogene on chromosome 7
-> chromosome 7 associated with papillary tumors

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

What renal tumor is associated with hereditary leiomyomatosis renal cell carcinoma / how can these patients be differentiated from HPRCC?

A

Papillary renal cell carcinoma

These patients will develop cutaneous leiomyomas (smooth muscle tumors), even in the uterus in females

HPRCC patients will have no other clinical manifestations than renal tumors

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

What is Birt-Hogg-Dube syndrome associated with?

A

Autosomal dominant tumor suppressor loss

  1. Fibrofolliculomas -> fibroblast tumors of hair follicles with skin tags
  2. Pneumothoraces - due to development of pulmonary blebs / cysts
  3. Increased risk for RCCs (especially chromophobe), oncocytomas
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the classic triad of RCC?

A

Hematuria, flank pain, and palpable mass

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

What paraneoplastic syndromes are most commonly associated with RCC?

A
  1. Polycythemia - due to production of EPO
  2. Cushing syndrome - due to ACTH secretion
  3. Hypercalcemia - PTHrP
  4. Hypertension - increased renin production
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is Stauffer syndrome? What lab test will be abnormal?

A

Non metastatic liver dysfunction paraneoplastic syndrome caused by RCC
-> due to release of IL-6

Liver function tests will be abnormal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Where does RCC commonly metastasize to? What can a left-sided RCC cause which a right-sided cannot?

A

Moves through IVC -> commonly goes to lung and bone

Left-sided = varicocele, due to compression of renal vein, which drains the testicular vein on the left but not the right

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

How does a clear cell renal carcinoma appear grossly?

A

Large tumors which are most often golden-yellow due to high lipid content

Tumors are often cystic, with frequent necrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Why do the cells appear clear in renal cell RCC? What will be the other impressive feature of it microscopically?

A

Cells are clear due to high amounts of glyogen and lipid which get washed out in staining

Impressive sinusoidal vascular pattern

20
Q

How is RCC staged?

A

Stage 1 - confined to kidney, less than 7 cm
Stage 2 - confined to kined, greater than 7 cm
Stage 3 - invaded into perinephric fat but not broken capsule (Gerota’s fascia)
Stage 4 - Invasion through capsule (including ipsilateral adrenal gland), or any distant metastases

21
Q

What is an angiomyolipoma? How does it present if acute?

A

Hamartoma with “Triphasic” morphology - blood vessels, smooth muscle, fat

Presents with hemorrhage (due to blood vessel, in sporadic form) or renal failure (genetic form)

22
Q

How does the presentation of angiomyolipoma differ between sporadic and genetic/hereditary-predisposed individuals?

A

Sporadic - usually solitary lesion -> usually presents with hemorrhage

Hereditary - happens in Tuberous sclerosis (disease of hamartomas) -> usually multiple and bilateral, which is why it causes renal failure

23
Q

What is the most common pediatric renal neoplasm? What cells is it comprised of?

A

Wilms tumor

Triphasic - Comprised of blastemal (primitive mesenchyme), epithelial (primitive tubules and glomeruli), and developed mesenchymal (stromal cells)

24
Q

Are the majority of Wilms tumors sporadic or inherited? What chromosome is at fault / why?

A

Vast majority are sporadic, but there are well known associated clinical syndromes
-> chromosome 11 in all these syndromes, which has WT1 and WT2 tumor suppressor genes

25
Q

What are the three syndromes associated with Wilms tumor? What gene is awry in each?

A
  1. WAGR syndrome - WT1
  2. Denys-Drash syndrome - WT1
  3. Beckwith-Wiedemann syndrome - WT2 (only one that is due to imprinting problem)
26
Q

What are the features of WAGR syndrome?

A

Wilms tumor
Aniridia
Genitourinary tract abnormalities - cryptorchism in males and streak gonads in females
R - Retardation (mental)

27
Q

What are the features of Denys-Drash syndrome?

A

Wilms tumor, early onset-nephrotic syndrome, male pseudohermaphroditism (gonads are testes, but secondary sex characteristics are female, i.e. 5-alpha hydroxylase deficiency)

28
Q

What are the features of Beckwith-Wiedemann?

A

Wilms tumor, macroglossia, organomegaly, hemihyperplasia (one side very large)

29
Q

What are the important risk factors for transitional cell bladder cancer?

A

Pee SAC

Phenacetin - discontinued NSAID
Smoking
Aniline dyes
Cyclophosphamide

30
Q

What are the important risk factors for squamous cell carcinoma of the bladder?

A

Schistosoma haematobium infection (especially Middle east)
Chronic cystitis (UTIs)
Smoking
Chronic nephrolithiasis (irritation conditions)

31
Q

What is the most common presenting symptom of bladder cancer and how are atypical cells first identified?

A

Painless hematuria, although there may be irritative or obstructive symptoms

Cells first identified by urine cytology

32
Q

What two procedures are used in diagnosis and treatment of bladder cancer?

A

Cystoscopy - Low risk, can even resect small papillary tumors

Transurethral resection of bladder tumor (TURBT) - diagnosis and staging, +treatment of non-muscle invasive bladder cancer

33
Q

What is the most common type of bladder cancer in North America and where do these cancers arise?

A

Transitional cell carcinoma (urothelial)

Arise anywhere from renal pelvis to urethra

34
Q

Are flat or papillary tumors more invasive and why?

A

Flat -> associated with early p53 mutations, early high grade tumor which invades bladder wall

Papillary -> low grade, NOT associated with early p53 mutations, grows outward and invades bladder wall very late

35
Q

Do urothelial tumors commonly arise as a solitary mass?

A

No -> associated with multifocality most often because of the field effect

36
Q

How does a papillary tumor of the bladder appear, and how do you know it is becoming higher grade?

A

Exophytic, frond-like appearance

Higher grade -> fused papillae, more solid, sessile appearance

37
Q

What will be seen microscopically with urothelial tumors?

A

Central fibrovascular cores covered by urothelium

-> increasing amounts of anaplasia as malignancy and grade increases

38
Q

How effective is TURBT resection at clearing urothelial tumors? When will patients ultimately need cystectomy?

A

About 50% of patients will develop another tumor after initial resection (probably due to field effect)

Patients will ultimately need cystectomy if the tumor invades the bladder muscle (invasion of muscularis propria)

39
Q

Is papillary urothelial carcinoma or de novo carcinoma in situ (flat carcinoma) more common?

A

Papillary is much more common -> this may progress to this invasive carcinoma in situ

De novo urothelial CIS is associated with early p53 mutations

40
Q

How does flat bladder urothelial carcinoma / CIS look grossly?

A

Hemorrhagic, erythematous, red

41
Q

How does flat bladder urothelial carcinoma look microscopically? How does this relate to what’s useful for diagnosis?

A

Partial or full thickness replacment of urothelium by cytologically malignant cells

Cells are “discohesive”, so urine will contain malignant cells -> urine cytology useful for diagnosis (malignant epithelial cells slough off easily)

42
Q

What is the staging system for bladder cancer?

A

Stage 1 - Invasion of submucosa, but not muscle layers
Stage 2 - Invasion of muscularis propria
Stage 3 - Invasion of peribladder tissues
Stage 4 - Invasion of adjacent structures (i.e. prostate, cervix)

43
Q

When do patients get squamous cell carcinoma of the bladder? What’s the prognosis?

A

Environments of chronic irritation and inflammation

  • Bladder stones
  • Indwelling catheteres
  • Typically older adults

-Schistosomiasis is endemic to Egypt (can happen in young adults)

Poor prognosis

44
Q

What is the most common cause of primary bladder adenocarcinoma? Where do they appear?

A

Arise from urachal remnant and are mucinous tumors which arise in dome or posterior wall of bladder

-> detected via mucinuria

Urachus is duct which attaches fetal bladder to umbilicus

45
Q

What is cystitis glandularis?

A

Columnar metaplasia of cells of the bladder

46
Q

What is the most common bladder tumor in kids?

A

Rhabdomyosarcoma - an embryonic small blue cell tumor

47
Q

How do rhabdomyosarcomas grow?

A

Gelatinous polyploid masses called “sarcoma botryoides” -> botryoides means grape-like

Pathophysiology - Urinary Tract (14 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions