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Nephrology > Renal Neoplastic Disease > Flashcards

Renal Neoplastic Disease Flashcards

1
Q
  1. Renal cell carcinoma (RCC) is cancer of the kidney that originates in the?
  2. RCC represents____% of primary renal neoplasms

RCC represents __% of overall cancer incidence and mortality

A
  1. renal cortex
  2. 80-85%
  3. 2
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2
Q

Epidemiology:

  1. What gender is RCC more common in?
  2. Highest incidence between age___ and ____?
  3. Median age of diagnosis is ___ years
  4. Median age of death___ years
A
  1. Male predominance: RCC is approximately 50% more common in men than women
  2. 60-80
  3. 64
  4. 70
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3
Q

Renal cell carcinoma risk factors?

4

A
  1. Smoking 1/3 of cases
  2. Hypertension
  3. Obesity
  4. Acquired cystic disease of the kidney

Usually an incidental finding

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4
Q

Renal Cell Carcinoma SubTypes

5

A
  1. Clear Cell (75 to 85 %)
  2. Papillary (chromophilic) (10 to 15%)
  3. Chromophobe (5 to 10%)
  4. Oncocytic (3 to 7%)
  5. Collecting duct (Bellini’s duct) (very rare)
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5
Q

Subtypes of renal cell carcinoma
Clear cell carcinomas (75-85%)
1. Arises from where?

  1. Whats the genetic variation that is associated with this?
  2. Macroscopic characteristics? 2
  3. Tends to invade where?
A
  1. arise from the proximal tubule
  2. typically have a deletion of chromosome 3p
  3. Macroscopically, they may be solid or less commonly cystic.
  4. Tends to have a vascular invasion
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6
Q

Variants of renal cell carcinoma
Papillary (“chromophilic”) carcinoma (familial or sporadic) (10-15%)
1. Thought arise from where?

  1. Genetic variation associated with this?
  2. Divided into what?
  3. How would you describe the prognosis?
A
  1. Thought to arise from proximal convoluted tubule
  2. Trisomy 7 common (also trisomy 16, 17, loss of Y)
  3. Divided into Type 1 and Type 2
  4. Recent study shows better prognosis than clear cell carcinoma
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7
Q

Chromophobe renal cell carcinoma
1. Compare this histologically with clear cell carcinoma

  1. Arises from where?
  2. Prognosis compared to clear cell carcinoma?
A
  1. Histologically, composed of sheets of cells that are darker than clear cell carcinoma
  2. Arises from intercalated cells of collecting ducts
  3. lower risk of disease progression and death compared to clear cell carcinomas
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8
Q

Collecting duct carcinoma (Bellini duct carcinoma)

  1. How prevelent is this?
  2. Describe the clinical course?
  3. In what demographics most often? 2
A
  1. Approximately 1% of renal cell carcinomas
  2. Aggressive clinical course
  3. Affects younger adults and African American patients more frequently
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9
Q

Oncocytomas (3-7%)
1. Where do these orginiate?

  1. What so they consist of?
  2. Genetic variation associated with this?
  3. Tends to be unilateral or bilateral? Single or multiple?
A
  1. originate from the intercalated cells of the collecting ducts
  2. consist of oncocytes, which are large well-differentiated neoplastic cells with intensely eosinophilic granular cytoplasm
  3. Mutations in c-met oncogene associated with papillary RCC
  4. Tends to be unilateral and single, but multiple and bilateral oncocytomas have been described
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10
Q

Renal Cell Carcinoma: Microscopic Appearances (1)
Clear Cell Carcinoma:
1. Describe the cytoplasm?

  1. Describe the cells? 2
  2. What do the cells form? 3
A
  1. Clear cytoplasm due to lipid and glycogen;
  2. cells may be
    - bland or
    - poorly differentiated;
  3. cells form
    - solid nests,
    - tubules;
    - fine vasculature
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11
Q

Renal Cell Carcinoma: Microscopic Appearances (2)
Papillary carcinoma:
1. Papillae are composed of what?

  1. Papillary tips may undergo what?
A

Papillary carcinoma
1. delicate vascular cores with overlying layer of tumor cells

  1. Papillary tips may undergo necrosis and calcification (“psammoma bodies”)
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12
Q

Renal Cell Carcinoma: Microscopic Appearances (3)

Chromophobe renal cell carcinoma?

A

Sheets of pink (eosinophilic) cells with perinuclear halos

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13
Q

Renal Cell Carcinoma: Clinical Features:
1. Presents when in the course of the disease?

  1. Classic Presentation** (classical triad)?
  2. Nonspecific features? 2
  3. Other features that could be associated? 3
A
  1. Presents late in course and most commonly diagnosed incidentally

2.

  • Flank pain (constant dull ache, back or abd)
  • Gross hematuria
  • Palpable renal mass
  1. -Sudden onset of a scrotal varicocele
    -Paraneoplastic syndromes
    due to ectopic production of various hormones
  2. Anemia
  3. Hepatic dysfunction
  4. Anorexia
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14
Q

Renal Cell Carcinoma diagnostic evaluation?

3

A

Diagnostic Evaluation:

  1. Ultrasound - initial
  2. CT Scan - gold standard
  3. MRI
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15
Q

Differential Diagnosis of Renal Masses

A 
Cysts
  -Simple
  -Complex
  -Multiple
Bosniak Scale
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16
Q

Ultrasound:

3 Major Criteria for simple cyst?

A
  1. Mass is round and sharply demarcated with smooth walls
  2. No echoes (anechoic) within the mass
  3. Strong posterior wall echo indicating good transmission through the cyst
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17
Q

The primary reason to investigate a renal mass is to do what?

A

exclude a malignant neoplasm

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18
Q

Renal Cell Carcinoma
Assessing for Metastasis
Most common sites of metastatic disease include?
3

A
  1. lung
  2. lymph nodes (intra abdominal)
  3. bone
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19
Q

Renal Cell Carcinoma
Assessing for Metastasis
3

A
  1. Bone scan
  2. CT of the chest
  3. Positron Emission Tomography (PET)
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20
Q

What is the treatment of choice for renal cell carcinoma?

A

surgical removal

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21
Q
  1. What is the surgery that we would do for renal cell carcinoma?
  2. How often are the regional lymphnodes involed?
  3. What else is involved in 5% of pts?
A
  1. Nephrectomy
  2. almost 25% of patients
  3. inferior vena cava involvement
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22
Q

The role of radiotherapy remains controversial in the treatment of renal cell carcinoma. Why?

A

Renal cell carcinoma is a relatively radioresistant tumor

Chemotherapy, Immunotherapy have limited role in tx

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23
Q

SOOOO what is the gold standard for treatment of renal cell carcinoma?

A

Radical nephrectomy

-The “gold standard” therapy for RCC

24
Q

A radical nephrectomy involves the ligation, dissection or removal of what structures?
5

A

Includes

  1. early vascular ligation
  2. extrafascial dissection of the kidney (outside Gerota’s fascia),
  3. en bloc resection of the adrenal gland
  4. extensive lymphadenectomy from the crus of the diaphragm to the aortic bifurcation.
  5. Resection of tumor extension into the Inferior Vena Cava
25
Q

Nephron-Sparing Surgery (Partial Nephrectomy)
Recently, several long-term studies have shown equivalent survival of patients undergoing NSS versus radical nephrectomy for a unilateral renal tumor less than ___ cm in size

A

4

26
Q

Nephron-Sparing Surgery (Partial Nephrectomy):
The risk of recurrence increases with what?
5

A
  1. larger tumors
  2. bilateral tumors
  3. multifocality
  4. symptoms
  5. certain histologies such as papillary RCC
27
Q

Stage I renal cysts are characterized how?

5 yr survival rate?

A

Tumor less than 7 in greatest dimension and limited to kidney.

5 yr survival 95%

28
Q

Stage II renal cysts are characterized how?

5 yr survival rate?

A

Tumor greater than 7 cm in greatest dimension and limited to kidney.

5 yr survival - 88%

29
Q

Stage II renal cysts are characterized how?

5 yr survival rate?

A

Tumor in major veins or adrenal gland, tumor with in Gerota’s fascia or 1 regional lympth node involved

5 yr survival 59%

30
Q

Stage III renal cysts are characterized how?

5 yr survival rate?

A

Tumor beyound Gerota’s fascia or > 1 regional lymph node involved.

5 year survival 20%

31
Q

Wilms’ Tumor (Nephroblastoma)
is found in who?

Whats the mean age?

A

Pediatric Tumor
The most common renal malignancy in children

3.5 yrs

32
Q

Wilms’ Tumor (Nephroblastoma)
pathogenesis?
(caused by and resulting in?)

A
  1. Caused by abnormal renal development,

2. resulting in proliferation of the metanephric blastema without normal tubular and glomerular differentiation.

33
Q

Wilms’ Tumor (Nephroblastoma)
Associated with congenital syndromes (rare) such as?
3

A
  1. WAGR syndrome
  2. Denys-Drash syndrome
  3. Beckwith-Wiedemann syndromes
34
Q

Wilms’ Tumor (Nephroblastoma)
risk factors?
4

A
  1. Aniridia
  2. Hemihypertrophy
  3. Undescended testicles
  4. Hypospadias
35
Q

Wilms’ Tumor: Clinical Features

5

A
  1. Palpable mass in 90% of cases
    - -May be large, extending into pelvis
  2. Abdominal pain in 30%
  3. Hematuria 12-25%
  4. Hypertension in 25%
  5. Bilateral 5-10%
36
Q

Wilms’ Tumor (Nephroblastoma)
diagnositics?
2

A

Radiologic imaging including

  1. abdominal US and
  2. contrast-enhanced CT

is useful to differentiate Wilms tumor from other causes of abdominal masses.

37
Q
  1. Wilms’ Tumor (Nephroblastoma)
    The definitive diagnosis of Wilms tumor is made by what?
  2. What is staging based on?
A
  1. histologic confirmation, either at the time of surgical excision or by biopsy.
  2. Staging is based upon the anatomic extent of the tumor without consideration for genetic, histologic, or biological markers.
38
Q

Wilms’ Tumor (Nephroblastoma) Staging

Describe stages I-V?

A
  1. Stage I: Cancer is found in one kidney only and can be completely removed by surgery.
  2. Stage II: Cancer has spread beyond the kidney to the surrounding area, but can be completely removed by surgery.
  3. Stage III: Cancer has not spread beyond the abdomen, but cannot be completely removed by surgery.
  4. Stage IV: Cancer has spread to distant parts of the body; most commonly, the lungs, liver, bone, and/or brain.
  5. Stage V: Cancer is found in both kidneys at diagnosis (also called bilateral tumors).
39
Q

Wilms’ Tumor: Macroscopic Appearance
(size, differentiation, color, cystic?)
3

A
  1. Usually large single well-circumscribed
  2. Tan/white, not as much hemorrhage as RCC
  3. Occasional cyst formation
40
Q

Wilms’ Tumor (Nephroblastoma)
Treatment?
2

A

Treatment decisions and evaluation of long-term outcome are based upon the Wilms tumor stage.

  1. Surgery for stages I-IV
  2. Chemo and Radiation for Stage V

These kids need to get on a transplant list

41
Q

Benign renal tumors?
3

Other malignant renal tumors? 2

A
  1. Papillary adenoma
  2. fibroma/hamartoma
  3. Angiomyolipoma
  4. Transitional Call Carcinoma
  5. Renal sarcomas
42
Q

“Benign” Renal Tumors: Renal papillary adenoma.

  1. Arises from where?
  2. Histolgically similar to what?
  3. Genetic varation?
  4. How should we treat it?
A

Renal Papillary “Adenoma”

  1. Arise from cortical renal tubules
  2. Histologically similar to low-grade papillary cancers
  3. Usually contain trisomy 7 and 17
  4. No unequivocal “benign” marker, treat as early cancer (including tumors
43
Q

What is a renal fibroma/hamartoma?

A

Small benign fibrous nodules in renal pyramids

44
Q

Benign renal tumor:
Angiomyolipoma
1. Benign tumor composed of what?
2. Present in up to half of pts with what?
3. Why is it easy to spot radiographically?

A
  1. Benign tumor composed of
    - blood vessels (angio)
    - smooth muscle (myo)
    - adipose tissue (lipoma)
  2. Present in up to half of patients with tuberous sclerosis
  3. Easy to spot radiographically because of fat content
45
Q

Transitional cell carcinoma of the renal pelvis:

  1. Where does it arse from?
  2. Peak incidence is in who?
  3. What gender more?
  4. Race?
A
  1. A malignant tumor arising from the transitional epithelial cells that line the urinary tract from renal calyces to ureteral orifices.
  2. Peak incidence 60-70 y/o
  3. Men 2x more than women
  4. Incidence slightly higher in African Americans
46
Q

TCC presentation?
2

TCC workup?
3

A

Presentation:

  1. Hematuria
  2. Renal colic

Workup:

  1. Urine cytology
  2. Cystoscopy
  3. CT IVP
47
Q

TCC treatment?

2

A
  1. Radical nephroureterectomy

2. Chemo

48
Q
  1. What is a Renal sarcoma?
  2. Peak in what decade of life?
  3. Clinical presentation? 3
A
  1. Rare malignant tumor derived from the mesenchymal cells
  2. Peak in 5th decade of life
  3. Presentation
    - Abdominal pain
    - Palpable mass
    - Gross hematuria
49
Q

Renal sarcoma diagnostics? 2

Treatment? 1

A

Diagnostics
-CT and MRI

Treatment
-Surgical excision

50
Q
  1. What are the most common primary tumor arising in the kidney, accounting for approximately 80-85%?
A
  1. RCCs
51
Q
  1. The most common histologic pattern of RCC is what?
  2. The incidence of RCC has been steadily increasing in the US in part, as a consequence of increased use of what?
  3. Risk factors associated with a significantly increased incidence of RCC include what? 3
A
  1. clear cell
  2. imaging procedures for other reasons
    • smoking,
    • obesity,
    • hypertension.
52
Q

RCC can present with a range of symptoms due what? 4

Ultrasound and CT can

  1. confirm What?
  2. distinguish between what?
  3. Assess what?
A
  1. tumor itself (mass, pain)
  2. invasion of the urinary tract (hematuria),
  3. paraneoplastic syndromes
  4. the presence of metastases.
  5. presence of a mass,
  6. RCC from a benign cyst
  7. the extent of disease
53
Q
  1. For pts with a resectable stage I, II, or III RCC, what is the primary treatment?
  2. _____ _________ has been the most widely used approach and remains the preferred procedure when there is evidence of invasion into the adrenal, renal vein, or perinephric fat.
A
  1. surgery is the primary treatment

2. Radical nephrectomy

54
Q

_____ ________ is an alternative for smaller tumors and is particularly valuable in pts with bilateral or multiple lesions, and those with what?

A
  1. Partial Nephrectomy

2. impaired renal function.

55
Q

_____ ______is the most common renal malignancy in children and the fourth most common childhood cancer.
Almost all cases are diagnosed before___ years of age

The most common presentation is what? 2

A

Wilms tumor

10

  1. detection of an abdominal mass or
  2. swelling without other signs or symptoms.
56
Q

Children who are suspected of having Wilms tumor should be referred to a _____ ______ ______ for management.

Treatment decisions and evaluation of long-term outcome are based upon the _____ _____ _____?

A

pediatric cancer center

Wilms tumor stage.

57
Q

BIggest take home point on symtpoms of renal cancer/tumors?

2

A
  1. NEVER IGNORE HEMATURIA- 2. always follow-up on a positive urinalysis

Nephrology (15 decks)

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