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68 Cards in this Set
- Front
- Back
Wilms Tumor (Nephroblastoma) is pediatric or adult?
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ped
blast=immature |
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Malignant pediatric tumor of the kidneys comprised of embryonal nephrogenic elements
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Wilms Tumor (Nephroblastoma)
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Most common malignant pediatric kidney tumor?
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Wilms Tumor (Nephroblastoma)
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why is a Wilms tumor called triphasic?
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has 3 types of tissue
Blastema: small densely packed cells with little cytoplasm; grow in nests or cords (trabeculae) Epithelium: abortive tubules and glomeruli Stroma: spindle cells of varying differentiation (Undifferentiated; skeletal muscle (most common), cartilage) |
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a triphasic neoplasm in kids =
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Wilms Tumor (Nephroblastoma)
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Blastema =?
seen in? |
small densely packed cells with little cytoplasm; grow in nests or cords (trabeculae)
Wilms Tumor |
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Blastema, epithelium, and stomal cells are seen in what?
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Wilms tumor
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unilateral abdominal tumor in a child =
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Wilms tumor
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What is the rule of 5 in tumors? what population is it found in? what specific tumor is this with?
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Accounts for ~ 5% of childhood cancers
5% bilateral 5% multicentric 5% anaplastic (histopathologic feature) – worse prognosis in kids Wilms tumor |
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85% of pediatric renal neoplasms =
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Wilms tumor
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Wilms tumors metastasize to where?
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Lung
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What are the 3 associated symptoms with Wilms tumor?
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Beckwith-Wiedeman (organomegaly, hemihypertrophy)
WAGR syndrome (Wilms-aniridia-genital anomaly-retardation) Denys-Drash syndrome (Glomerulonephritis, male gonadal dysgenesis) 1st two are most common |
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Prognosis for Wilms tumor?
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if Anaplasia is present--> poor prognostic
yet 80-90% overall cure rate |
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tx for wilms tumor?
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nephrectomy plus chemo
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Pathogenesis of Wilms tumor?
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WT1 gene protein regulates transcription of growth-promoting genes (e.g., IGF-2)
WT2 gene product also interacts with growth promoting factors both on chromosome 11 and do growth promoting factors |
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classic histology in Wilms tumor?
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triphasic microscopic appearance:
Primitive tubules (blue arrows) Blastema (red arrows) Stroma (green arrows) |
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immature islands of tissue and small tubules of tissue and spindle areas =
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triphasic microscopic appearance found in
Wilms tumor |
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Most common renal tumor in INFANCY (less than 1 year)
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Congenital Mesoblastic Nephroma
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palpable mass, spindle cells, and immature tissues found in an infant =
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Congenital Mesoblastic Nephroma
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discuss metastases in Congenital Mesoblastic Nephroma
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they are RARE
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majority of adult renal neoplasms are derived from where?
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epithelial components
aka tubular cells |
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what are the 3 benign neoplasms we need to know
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papillary adenoma
Renal oncocytoma Angiomyolipoma |
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Key diagnostic feature of a papillary adenoma?
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SIZE at time of diagnosis
≤0.5 cm = adenoma; >0.5 cm = papillary renal cell carcinoma |
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how do you define the difference btw a benign and malignant papillary neoplasm
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.5 cm < =Papillary adenoma--> benign
.5 cm> = Papillary carcinoma -->aggressive |
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Most common renal tubular epithelial neoplasm?
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Renal papillary adenoma
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what is normally found in acquired cystic renal disease?
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Renal papillary adenoma
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What can be a long term result of hemodialysis?
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acquired cystic renal disease --> renal papillary adenoma
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Higher power view: fibrovascular cores lined by bland neoplastic cells with uniform, round to oval nucli =
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papillary adenoma
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Well circumscribed, tan/yellow/mahogany brown =
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Renal Oncocytoma
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Central stellate scar =
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Renal Oncocytoma
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chromophobe variant is a DD for ?
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Renal Oncocytoma
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large tumor with lots of mitochondria that can be seen in other places =
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Oncocytoma
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solid, mesenchymal tumor that is benign =
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angiomyolipoma
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what are the 3 tissue types in a AML?
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blood
muscle fat |
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Tuberous sclerosis and von Recklinghausen disease (NF1) are associated with what?
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Angiomyolipoma
these are both HEREDITARY disorders |
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although benign, fatality may result in Angiomyolipoma from.....
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massive hemorrhage
renal failure from significant loss of functional renal parenchyma |
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if you see a multifocal Angiomyolipoma what should you be thinking
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underlying hereditary component---> tuberous sclerosis
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LM finding of mixture of mature adipose tissue, spindled cells with smooth muscle features, and thick-walled blood vessels
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Angiomyolipoma
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most common malignant renal neoplasm in adults =
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Renal cell carcinoma (RCC)
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most common Renal cell carcinoma (RCC)
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conventional
or clear cell |
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malignant tumor made of transitional cells involving the collecting system
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RCC
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2/3 are men; >50 yo; only ~ 1% are bilateral
Bilaterally strongly suggestive of underlying syndrome |
RCC
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biggest risk factor for RCC?
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SMOKING
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von Hippel Lindau disease can be a risk factor for what?
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RCC
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von Hippel Lindau disease =
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found in RCC
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classic triad seen in RCC?
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Flank pain
palpable mass hematuria |
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Pt is in a car wreck and has suspected internal bleeding, upon imaging what tumor would likely be found?
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RCC
most commonly found incidentally |
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25% of the time what is found at time of presentation for RCC?
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metastases
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5 year survival for RCC
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70%
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What can help determine overall 5 year survival? (2)
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tumor stage and nuclear grade
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How does chemo help in RCC?
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it doesn't really
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major tx of RCC?
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surgical resection
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genetics of RCC?
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VHL gene (3p25) – mutation at this locus seen in >90% of cases
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what does mutation of VHL do? in what cancer?
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VHL is part of the ubiquitin ligase complex which is used to target proteins for degradation.... so if you mess this up, you lose the ability to break down protein (aka lose tumor suppressant)
Clear Cell RCC |
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high levels of VFEG is seen in what?
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clear RCC
because lack of VHL gene will lead to a highly vascular tumor |
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what is TNM in tumor staging?
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T – size of primary tumor; extent of invasion beyond kidney
N – presence of metastases to regional lymph nodes M – distant metastasis |
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cyctoplasm clear with glycogen =
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Clear cell RCC
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germline mutation in c-met gene =
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Papillary RCC
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Associated with ESRD (“dialysis kidneys”)
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Papillary RCC
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More often multiple than any other type of RCC
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Papillary RCC
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size of a Papillary RCC
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> 0.5 cm
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which has a better prognosis Papillary RCC or clear?
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Papillary RCC
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Rare RCC with worst prognosis of subtypes. Typically presents as painless gross hematuria
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Collecting Duct Carcinoma
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A Mediterranean man presents with sickle-cell trait and a renal mass. What does he have
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Renal Medullary Carcinoma
could be a black dude too |
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Neoplasms of transitional cell epithelium (TCC) =
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Renal Urothelial Carcinomas
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this is multifocal in development and undergoes the carcinogenic "feild effect" from tobacco
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Renal Urothelial Carcinomas
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adult man presents, found to have blood in the urine (microscopic) that he didn't know about (aka incidental), he has some microcytic hemolytic anemia. What does he have?
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RCC
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adult man presents, found to have blood in the urine (microscopic) that he didn't know about (aka incidental), he has some microcytic hemolytic anemia. What would be a genetic finding?
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VHL mutation
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