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108 Cards in this Set
- Front
- Back
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What is the most common form of renal tumor?
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Renal cell carcinoma
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What is the typical age of onset of RCC?
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50-70
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What is the gender distribution of RCC?
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M:F 2:1
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What is the origin of the tumor cells in RCC?
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Renal tubular epithelium (adenocarcinoma)
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Tobacco
Obesity HTN Unopposed estrogen Chronic dialysis for CRF & acquired cystic dz Heavy metal (cadmium), petroleum, asbestos Genetics These are risk factors for what? |
RCC
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What is the "classic triad" of RCC?
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Gross hematuria
Flank pain Mass |
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What is the most common symptom of RCC?
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Painless hematuria
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What are the paraneoplastic syndromes related to RCC?
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Polycythemia
HTN Hypercalcemia Hyponatremia Cushings |
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HTN in RCC is due to what?
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Increased renin
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Hypercalcemia in RCC is due to what?
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Increased PTH-like protein
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Hyponatremia in RCC is due to what?
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Increrased ADH
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What are some rare paraneoplastic syndromes associated w/RCC?
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Hepatic dysfunction
Feminization/masculinization Amyloidosis Leukemoid reaction |
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RCC that has invaded the perirenal fat is at what stage?
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pT3a
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RCC that grossly extends in renal vein is at what stage?
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pT3a
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RCC limited to the kidney that is 5cm is at what stage?
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pT1b
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RCC that has reached the adrenal cortex is at what stage?
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pT4
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An RCC has 15UM nuclei and the nucleoli are not easily visible at 10x. What grade is this?
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G2
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An RCC has 20UM nuclei and the nucleoli are easily visible at 10x. What grade is this?
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G3
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What are the treatment options for RCC?
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Nephrectomy
Radiation & chemo IL2 |
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What is the most common kind of RCC?
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CCC
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What RCC is described as having solid to cord-like arrangements and abundant clear cytoplasm with thin vasculature throughout the tumor?
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CCC
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What mutation is associated with CCC?
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Loss of chromosome 3p
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What syndrome is associated with CCC?
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Von Hippel Lindau
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What protein does VHL gene code for?
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protein which is part of a ubiquitin degradation complex
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What mutations are associated with papillary RCC?
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Trisomies of chromosomes 7 and 17
Mutations in MET on chromosome 7 |
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A mutation on chromosome 7 is associated with what kind of tumor and what protein?
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Papillary RCC
MET is a proto-oncogene encoding a growth factor |
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What type of RCC has a better prognosis?
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Chromophobe
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What is the morphology of chromophobe RCC?
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Prominent cell borders like CCC but with thicker cell membranes & perinuclear halos
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What is the hereditary pattern of Von Hippel-Lindau syndrome?
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AD
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What is the mutation responsible for Von Hippel-Lindau syndrome?
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Unbalanced translocation of VHL gene 3p25-26 bilaterally
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What tumors are associated w/Von Hippel-Lindau syndrome?
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RCC (usually CCC)-->50% get RCC
Also: pheochromocytoma, retinal angioma, hemangioblastoma |
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A translocation on chromosome 3 makes you worry about a number of malignancies. What are they?
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CCC
Pheochromocytoma, retinal angioma, hemangioblastoma |
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Nephroblastoma is also known as what?
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Wilms' Tumor
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What is associated with Wilms tumor?
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Wilms tumor
Aniridia GU malformations Retardation |
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What other syndromes may be associated w/WAGR syndrome?
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Beckwith-Wiedermann
Deny-Drash |
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What is Beckwith-Wiedermann syndrome and what tumor is it associated with?
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Hemihypertrophy.
Wilms tumor |
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What is the morphology of Wilms' tumor?
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Triphasic tumor with spindle components, blasts, and glandular cells
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What is an angiomyolipoma?
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Benign tumor composed of fat, smooth muscle, and blood vessels
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What is associated with angiomyolipoma?
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Tuberous sclerosis
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What mutation is associated with tuberous sclerosis?
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TSC1 or TSC2 mutation
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What abnormalities are associated with tuberous sclerosis?
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Renal angiomyolipomas
Rhabdomyomas of the heart Cerebellar nodules Adenoma sebaceum Rarely RCCs |
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What is oncocytoma?
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Benign tumor
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What are the 4 layers of the bladder wall?
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Folded urothelium
Lamina propria Muscularis propria Adventitia |
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What is the most common bladder tumor?
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Urothelial cell carcinoma
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What are the demographics of urothelial cell carcinoma?
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Age 50-80, mostly 60s
M:F 3:1 |
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Smoking
Aromatic amines Rubber products Chronic cystitis Phenacetin Cyclophosphamide Radiation These are risk factors for what cancer? |
Urothelial cell carcinoma
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List the clinical features of urothelial cell carcinoma.
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Painless hematuria
Dysuria Urinary urgency & frequency Flank pain Metastatic disease |
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What is the morphology of UCC?
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Can be flat or papillary
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What is a flat low grade UCC also called?
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Carcinoma in situ
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What is the difference between flat & papillary UCC?
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Papillary simply has the tumor cells wrapped around a core.
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Is a papilloma malignant?
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NO.
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What is the difference between low and high grade UCCs?
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High-grade UCCs have pleomorphic cells and high malignant potential.
Low-grade UCCs have minimal cellular atypia and increased number of cell layers |
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What is pTa?
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noninvasive UC
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What is pTis?
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CIS (flat low grade UCC)
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A UCC invades the subepithelial tissue. What stage is this?
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pT1
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A UCC invades the muscularis propria. What stage is this?
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pT2a if it invades only superficial 1/2 of the muscle
pT2b is it invades the deep 1/2 of the muscle |
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A UCC invades the perivesicle fat. What stage is this?
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pT3a if it is microscopic invasion
pT3b if it is macroscopic invasion |
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In UCC, what is the difference between pT4a and pT4b?
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pT4a=invasion of prostate stroma, uterus, vagina
pT4b=invasion of abdominal or pelvic wall |
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What is the treatment for a superficial low-grade UC?
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TUR
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What is the treatment for a flat non-invasive UC or pT1 UC?
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intravesicualr chemo/BCG
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What is the treatment for tumors of stage pT2 and above?
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Cystectomy
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What is the 5y survival of UC patients?
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57%
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Risk factors are smoking and S. Haematobium. What is this cancer?
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Squamous cell carcinoma
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What is the process of squamous cell carcinoma development?
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Squamous metaplasia-->dysplasia-->squamous cell carcinoma
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A tumor has keratin pearls & intracellular bridges. What is it?
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Squamous cell carcinoma
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Where would an adenocarcinoma of the bladder arise?
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Areas of intestinal metaplasia
Urachal remnant Urothelium |
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A tumor has signet ring appearance. What type of tumor is it?
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Adenocarcnioma
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ADPKD has a typical of age of onset in what decade?
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40s
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What are the signs & symptoms of ADPKD?
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renal mass, flank pain, HTN, polyuria, proteinuria, hematuria, renal stone, pyelonephritis
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What might present with a berry aneurysm?
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ADPKD
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Mitral valve prolapse is a concern with what type of kidney disease?
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ADPKD
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PKD1 is associated with what?
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85% of all ADPKD
PKD1 codes for polycystin-1 |
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Where is PKD1 expressed?
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Tubular epithelial cells
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What is a genetic indicator of severe disease in ADPKD?
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PKD1 mutation
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What is the function of PKD1?
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cell-cell/cell-matrix interaction
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Where is PKD2 located?
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4q21
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What is PKD2 and what is it associated with?
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Polycystin-2 gene
ADPKD |
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Where is PKD2 expressed?
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Renal tubules
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What does PKD2 function in?
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Calcium channels
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What is the current theory of pathogenesis of ADPKD?
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Mutations result in defects which ultimately result in disruption of normal calcium influx
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What is the treatment of choice for ADPKD?
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Renal transplant
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What is the morphology of ADPKD?
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Enlarged kidneys w/multiple cysts in cortex & medulla filled with blood or fluid
Cysts lined with flattened epithelial cells Adjacent parenchyma is atrophic |
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What is the age of onset of ARPKD?
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infancy-adolescence
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What may complicate a pregnancy by oligohydramnios?
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ARPKD
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What is a major feature of ARPKD?
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HTN in infants & adolescnect
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What causes portal HTN in infants?
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ARPKD
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What is the genetic basis of ARPKD?
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Mutations in PKHD1 at 6p21-p23
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Mutations in PKHD1 are associated with what?
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ARPKD
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What is the morphology of ARPKD?
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Smooth surface with enlarged kidneys
Small cysts in cortex & medulla CDs dilated perpendicular to surface Liver has cysts |
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What cystic disease also has liver cysts?
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ARPKD
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What is inherited cystic kidney disease has onset MAINLY in adolescence?
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Medullary cystic disease
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What is the least common of the inherited cystic diseases?
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Medullary cystic disease
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What inherited disease presents with small cystic kidneys and a normal liver?
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Medullary cystic disease
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What is the clinical presentation of medullary cystic disease?
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Polyuria, polydipsia, salt-losing nephropathy, uremia
Small cystic kidney normal liver |
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What is the mutation associated with medullary cystic disease?
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MCDK1 and MCDK2
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What is associated with MCDK1?
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Onset of medullary cystic disease in the 50-60s
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What is associated with MCDK2?
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Onset of medullary cystic disease in the 20-50s (mean 32)
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What is the treatment of choice for medullary cystic disease?
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Renal transplant
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An autopsy shows small kidneys with a granular surface and corticomedullary cysts. What might this patient have had?
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Medullary cystic disease
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What is the microscopic appearance of medullary cystic disease?
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Tubular BM disintegration
Tubular atrophy w/cystic dilation Interstitial fibrosis and chronic inflammatory cell infiltration |
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Small cysts are found in the renal papillae at their connections with the connecting ducts. What disease is this?
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medullary sponge kidney
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What is medullary sponge kidney associated with?
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hematuria
UTIs calculi (medullary nephrocalcinosis) |
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What is the origin of mulcystic renal dysplasia?
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Fetal development of cysts in conjunction of persistence of undifferentiated mesenchyme and immature tubules often with cartilage
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What is the most common cystic cause of abdominal mass in an infant?
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Multicystic renal dysplasia
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What is found in multicystic kidney disease that is fairly unique?
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cartilage
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What may cause cysts to form?
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Chronic dialysis
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What is the pathogenesis of cyst d/t chronic dialysis?
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Destruction of renal parenchyma by cysts containing fluid & oxalate crystals
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Simple renal cysts have few clinical manifestations. What is one?
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Incidental hematuria
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