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STEP 7: Renal Urinary Tract PATHOMA FA CORBETT > Renal and Urinary Tract Cancer PATHOMA > Flashcards

Renal and Urinary Tract Cancer PATHOMA Flashcards

1
Q

What is the clinical triad of RENAL CELL CARCINOMA?? What is the most common Sx within the clinical triad?

A

HEMATURIA (most common) + PALPABLE MASS + FLANK PAIN

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2
Q

Can RENAL CELL CARCINOMA result in paraneoplastic syndromes? If so, what are the specific types?

A

RENIN - HTN
EPO - Reactive Polycythemia
PTHrP - Hypercalcemia
ACTH - Cushing’s

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3
Q

How can RENAL CELL CARCINOMAs present with varicocele? Are the varicoceles bilateral or unilateral?

A

LEFT SIDE, not right side varicocele
RCC -> Commonly involves the renal vein
Only LEFT spermatic vein drains into L. renal vein -> Drains into IVC
RIGHT spermatic vein drains directly into IVC

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4
Q

What is the most common variant of RENAL CELL CARCINOMA? Where does it originate? Why does it stain the way it does?

A

CLEAR CELL TYPE
Originates from PCT epithelial cells
Microscopically has clear cytoplasm - Bec of HIGH GLYCOGEN AND LIPID ACCUMULATION - that gets dissolved during staining techniques leaving CLEAR spaces

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5
Q

Which tumor suppressor gene is lost in the pathogenesis of RENAL CELL CARCINOMA? What happens as a result?

A

VHL (3p) tumor suppressor gene ->
INCREASED IGF-1 = Growth
INCREASED HIF -> INCREASED VEGF/PDGF = Angiogenesis

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6
Q

What is the major risk factor for SPORADIC RENAL CELL CARCINOMA? Is this subtype usually bilateral or unilateral

A

ADULT (avg age 60) SMOKERS

SINGLE (unilateral) in the upper pole of the kidney

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7
Q

Which disease is associated with HEREDITARY RENAL CELL CARCINOMA? What is the inheritance pattern?

A

VON HIPPEL-LINDAU disease

Autosomal Dominant

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8
Q

VHL Disease increases the risk of which 3 conditions?

A
  1. RENAL CELL CARCINOMA
  2. HEMANGIOBLASTOMA of Cerebellum
  3. PHEOCHROMOCYTOMA
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9
Q

Which lymph nodes do RENAL CELL CARCINOMAs tend to spread into?

A

RETROPERITONEAL

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10
Q

What is the hallmark cell constituting the malignant tumor of WILM’S TUMOR?

A

BLASTEMA cells (primitive cells of the kidney mesenchyme)

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11
Q

What is the most common age population that gets WILM’S TUMOR?

A

CHILDREN - Avg age = 3yo

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12
Q

Do WILM’ S TUMOR pts generally present with a bilateral or unilateral mass? What are other clinical signs of Wilm’s tumor? (2)

A

UNILATERAL large flank mass

HTN + HEMATURIA

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13
Q

Are most cases of WILM’S TUMOR sporadic or syndromic?

A

SPORADIC (90%)

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14
Q

What are the 3 syndromes associated with WILM’S TUMOR?

A
  1. WAGR Syndrome
  2. DENYS-DRASH Syndrome
  3. BECKWITH-WIEDEMANN Syndrome
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15
Q

What is the clinical presentation of WAGR syndrome associated with WILM’S TUMOR?

A
  1. W- Wilm’s tumor, 2. A-Anidiria,

3. G-Genital abnormalities, 4. R- Motor and Mental retardation

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16
Q

What is the clinical presentation of DENYS-DRASH associated with WILM’S TUMOR?

A

Progressive renal (glomerular disease) + Wilm’s Tumor + Male pseudohermaphroditism

17
Q

What is the clinical presentation of BECKWITH WIEDEMANN syndrome associated with WILM’S TUMOR?

A

‘Wilm’s affects children, 1 large - hemihyper, large- tongue’

  1. Wilm’s tumor
  2. Neonatal HYPOGLYCEMIA
  3. Muscular Hemihypertrophy
  4. Organomegaly (particularly tongue)
18
Q

Which tumor suppressor gene is lost or mutated in the pathogenesis of WILM’S TUMOR?

A

WT1 (WAGR + DENYS-DRASH Syndromic Wilm’s tumor)

WT2 (BECKWITH WIEDEMANN Syndromic Wilm’s tumor)

19
Q

In which pts is there an increased frequency of ANGIOMYOLIPOMA?

A

TUBEROUS SCLEROSIS

20
Q

What are the 3 common malignant URINARY TRACT CARCINOMAS? Which is the most common?

A
  1. *UROTHELIAL (Transitional Cell) CARCINOMA - most common
  2. SQUAMOUS CELL CARCINOMA
  3. ADENOCARCINOMA
21
Q

Which organ of the lower urinary tract (renal pelvis, ureter, bladder, urethra) is most commonly involved in urinary tract cancers?

A

BLADDER

22
Q

What is the most common age population affected by UROTHELIAL TRANSITIONAL CELL CARCINOMA? What is the most common clinical Sx?

A

Older population

PAINLESS hematuria

23
Q

What are the risk factors of BLADDER CANCER? Which is the major one?
*In this case think UROTHELIAL TRANSITIONAL CELL CARCINOMA since that is the most common of the 3 bladder cancers.

A

‘Pee SAC’ - 2 drugs and 2 carcinogens
P- PHENACETIN
S- *Smoking, Schistosoma haematobium (NOT UROTHELIAL, squamous)
A- Analine dye metabolite (aromatic amines)
C- CYCLOPHOSPHAMIDE

24
Q

What are the two pathways of TRANSITIONAL CELL CARCINOMA development? Which is associated with EARLY p53 mutations?

A
  1. FLAT - Associated with Early p53 mutations

2. PAPILLARY - NOT associated with early p53 mut

25
Q

Are UROTHELIAL (TRANSITIONAL CELL CARCINOMAS) solitary or multifocal? Do they have a high chance of recurrence, and why?

A

MULTIFOCAL
HIGH CHANCE OF RECURRENCE
Both due to FIELD DEFECT - The entire urothelial lining of the lower urinary tract is defective, and high [carcinogens] for a long period of time covers a LARGE surface (Multifocal) + many spontaneous mutations (Recurrence)

26
Q

What is the general pattern of progression for the PAPILLARY PATH of UROTHELIAL (TRANSITIONAL CELL) CARCINOMAS?

A

PAPILLARY: LOW GRADE -> HIGH G -> INVASIVE
FLAT: HG -> INVASIVE

27
Q

What are the 3 risk factors of SQUAMOUS CELL CARCINOMA involving the BLADDER?

A
  1. CHRONIC CYSTITIS in OLDER WOMAN
  2. SCHISTOMA HAEMATOBIUM in EGYPTIAN MALE (middle-eastern)
  3. CHRONIC NEPHROLITHIASIS
    All of these conditions cause chronic inflammation -> Squamous METAPLASIA -> Squamous DYSPLASIA -> SCC
28
Q

What are the 3 risk factors of ADENOCARCINOMA involving the bladder? (Hint: 2 congenital conditions, 1 chronic)

A
  1. URACHAL REMNANT along DOME OF BLADDER - Incomplete URACHUS (duct connecting fetal bladder to yolk sac) involution -> Formation of remnant
  2. BLADDER EXSTROPHY - Congenital failure to form caudal anterior abdominal and bladder walls -> Exposes bladder surface to outside
  3. CYSTITIS GLANDULARIS -> Chronic bladder inflammation -> COLUMNAR dysplasia

STEP 7: Renal Urinary Tract PATHOMA FA CORBETT (7 decks)

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