Renal and Urinary Tract Cancer PATHOMA Flashcards
What is the clinical triad of RENAL CELL CARCINOMA?? What is the most common Sx within the clinical triad?
HEMATURIA (most common) + PALPABLE MASS + FLANK PAIN
Can RENAL CELL CARCINOMA result in paraneoplastic syndromes? If so, what are the specific types?
RENIN - HTN
EPO - Reactive Polycythemia
PTHrP - Hypercalcemia
ACTH - Cushing’s
How can RENAL CELL CARCINOMAs present with varicocele? Are the varicoceles bilateral or unilateral?
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
What is the most common variant of RENAL CELL CARCINOMA? Where does it originate? Why does it stain the way it does?
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
Which tumor suppressor gene is lost in the pathogenesis of RENAL CELL CARCINOMA? What happens as a result?
VHL (3p) tumor suppressor gene ->
INCREASED IGF-1 = Growth
INCREASED HIF -> INCREASED VEGF/PDGF = Angiogenesis
What is the major risk factor for SPORADIC RENAL CELL CARCINOMA? Is this subtype usually bilateral or unilateral
ADULT (avg age 60) SMOKERS
SINGLE (unilateral) in the upper pole of the kidney
Which disease is associated with HEREDITARY RENAL CELL CARCINOMA? What is the inheritance pattern?
VON HIPPEL-LINDAU disease
Autosomal Dominant
VHL Disease increases the risk of which 3 conditions?
- RENAL CELL CARCINOMA
- HEMANGIOBLASTOMA of Cerebellum
- PHEOCHROMOCYTOMA
Which lymph nodes do RENAL CELL CARCINOMAs tend to spread into?
RETROPERITONEAL
What is the hallmark cell constituting the malignant tumor of WILM’S TUMOR?
BLASTEMA cells (primitive cells of the kidney mesenchyme)
What is the most common age population that gets WILM’S TUMOR?
CHILDREN - Avg age = 3yo
Do WILM’ S TUMOR pts generally present with a bilateral or unilateral mass? What are other clinical signs of Wilm’s tumor? (2)
UNILATERAL large flank mass
HTN + HEMATURIA
Are most cases of WILM’S TUMOR sporadic or syndromic?
SPORADIC (90%)
What are the 3 syndromes associated with WILM’S TUMOR?
- WAGR Syndrome
- DENYS-DRASH Syndrome
- BECKWITH-WIEDEMANN Syndrome
What is the clinical presentation of WAGR syndrome associated with WILM’S TUMOR?
- W- Wilm’s tumor, 2. A-Anidiria,
3. G-Genital abnormalities, 4. R- Motor and Mental retardation
What is the clinical presentation of DENYS-DRASH associated with WILM’S TUMOR?
Progressive renal (glomerular disease) + Wilm’s Tumor + Male pseudohermaphroditism
What is the clinical presentation of BECKWITH WIEDEMANN syndrome associated with WILM’S TUMOR?
‘Wilm’s affects children, 1 large - hemihyper, large- tongue’
- Wilm’s tumor
- Neonatal HYPOGLYCEMIA
- Muscular Hemihypertrophy
- Organomegaly (particularly tongue)
Which tumor suppressor gene is lost or mutated in the pathogenesis of WILM’S TUMOR?
WT1 (WAGR + DENYS-DRASH Syndromic Wilm’s tumor)
WT2 (BECKWITH WIEDEMANN Syndromic Wilm’s tumor)
In which pts is there an increased frequency of ANGIOMYOLIPOMA?
TUBEROUS SCLEROSIS
What are the 3 common malignant URINARY TRACT CARCINOMAS? Which is the most common?
- *UROTHELIAL (Transitional Cell) CARCINOMA - most common
- SQUAMOUS CELL CARCINOMA
- ADENOCARCINOMA
Which organ of the lower urinary tract (renal pelvis, ureter, bladder, urethra) is most commonly involved in urinary tract cancers?
BLADDER
What is the most common age population affected by UROTHELIAL TRANSITIONAL CELL CARCINOMA? What is the most common clinical Sx?
Older population
PAINLESS hematuria
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.
‘Pee SAC’ - 2 drugs and 2 carcinogens
P- PHENACETIN
S- *Smoking, Schistosoma haematobium (NOT UROTHELIAL, squamous)
A- Analine dye metabolite (aromatic amines)
C- CYCLOPHOSPHAMIDE
What are the two pathways of TRANSITIONAL CELL CARCINOMA development? Which is associated with EARLY p53 mutations?
- FLAT - Associated with Early p53 mutations
2. PAPILLARY - NOT associated with early p53 mut
Are UROTHELIAL (TRANSITIONAL CELL CARCINOMAS) solitary or multifocal? Do they have a high chance of recurrence, and why?
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)
What is the general pattern of progression for the PAPILLARY PATH of UROTHELIAL (TRANSITIONAL CELL) CARCINOMAS?
PAPILLARY: LOW GRADE -> HIGH G -> INVASIVE
FLAT: HG -> INVASIVE
What are the 3 risk factors of SQUAMOUS CELL CARCINOMA involving the BLADDER?
- CHRONIC CYSTITIS in OLDER WOMAN
- SCHISTOMA HAEMATOBIUM in EGYPTIAN MALE (middle-eastern)
- CHRONIC NEPHROLITHIASIS
All of these conditions cause chronic inflammation -> Squamous METAPLASIA -> Squamous DYSPLASIA -> SCC
What are the 3 risk factors of ADENOCARCINOMA involving the bladder? (Hint: 2 congenital conditions, 1 chronic)
- URACHAL REMNANT along DOME OF BLADDER - Incomplete URACHUS (duct connecting fetal bladder to yolk sac) involution -> Formation of remnant
- BLADDER EXSTROPHY - Congenital failure to form caudal anterior abdominal and bladder walls -> Exposes bladder surface to outside
- CYSTITIS GLANDULARIS -> Chronic bladder inflammation -> COLUMNAR dysplasia
