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68 Cards in this Set

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Wilms Tumor (Nephroblastoma) is pediatric or adult?
ped

blast=immature
Malignant pediatric tumor of the kidneys comprised of embryonal nephrogenic elements
Wilms Tumor (Nephroblastoma)
Most common malignant pediatric kidney tumor?
Wilms Tumor (Nephroblastoma)
why is a Wilms tumor called triphasic?
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)
a triphasic neoplasm in kids =
Wilms Tumor (Nephroblastoma)
Blastema =?

seen in?
small densely packed cells with little cytoplasm; grow in nests or cords (trabeculae)

Wilms Tumor
Blastema, epithelium, and stomal cells are seen in what?
Wilms tumor
unilateral abdominal tumor in a child =
Wilms tumor
What is the rule of 5 in tumors? what population is it found in? what specific tumor is this with?
Accounts for ~ 5% of childhood cancers
5% bilateral
5% multicentric
5% anaplastic (histopathologic feature) – worse prognosis


in kids

Wilms tumor
85% of pediatric renal neoplasms =
Wilms tumor
Wilms tumors metastasize to where?
Lung
What are the 3 associated symptoms with Wilms tumor?
Beckwith-Wiedeman (organomegaly, hemihypertrophy)

WAGR syndrome (Wilms-aniridia-genital anomaly-retardation)

Denys-Drash syndrome (Glomerulonephritis, male gonadal dysgenesis)

1st two are most common
Prognosis for Wilms tumor?
if Anaplasia is present--> poor prognostic

yet 80-90% overall cure rate
tx for wilms tumor?
nephrectomy plus chemo
Pathogenesis of Wilms tumor?
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
classic histology in Wilms tumor?
triphasic microscopic appearance:

Primitive tubules (blue arrows)
Blastema (red arrows)
Stroma (green arrows)
immature islands of tissue and small tubules of tissue and spindle areas =
triphasic microscopic appearance found in

Wilms tumor
Most common renal tumor in INFANCY (less than 1 year)
Congenital Mesoblastic Nephroma
palpable mass, spindle cells, and immature tissues found in an infant =
Congenital Mesoblastic Nephroma
discuss metastases in Congenital Mesoblastic Nephroma
they are RARE
majority of adult renal neoplasms are derived from where?
epithelial components

aka tubular cells
what are the 3 benign neoplasms we need to know
papillary adenoma

Renal oncocytoma

Angiomyolipoma
Key diagnostic feature of a papillary adenoma?

****
SIZE at time of diagnosis

≤0.5 cm = adenoma; >0.5 cm = papillary renal cell carcinoma
how do you define the difference btw a benign and malignant papillary neoplasm
.5 cm < =Papillary adenoma--> benign

.5 cm> = Papillary carcinoma -->aggressive
Most common renal tubular epithelial neoplasm?
Renal papillary adenoma
what is normally found in acquired cystic renal disease?
Renal papillary adenoma
What can be a long term result of hemodialysis?
acquired cystic renal disease --> renal papillary adenoma
Higher power view: fibrovascular cores lined by bland neoplastic cells with uniform, round to oval nucli =
papillary adenoma
Well circumscribed, tan/yellow/mahogany brown =
Renal Oncocytoma
Central stellate scar =
Renal Oncocytoma
chromophobe variant is a DD for ?
Renal Oncocytoma
large tumor with lots of mitochondria that can be seen in other places =
Oncocytoma
solid, mesenchymal tumor that is benign =
angiomyolipoma
what are the 3 tissue types in a AML?
blood

muscle

fat
Tuberous sclerosis and von Recklinghausen disease (NF1) are associated with what?
Angiomyolipoma


these are both HEREDITARY disorders
although benign, fatality may result in Angiomyolipoma from.....
massive hemorrhage

renal failure from significant loss of functional renal parenchyma
if you see a multifocal Angiomyolipoma what should you be thinking
underlying hereditary component---> tuberous sclerosis
LM finding of mixture of mature adipose tissue, spindled cells with smooth muscle features, and thick-walled blood vessels
Angiomyolipoma
most common malignant renal neoplasm in adults =
Renal cell carcinoma (RCC)
most common Renal cell carcinoma (RCC)
conventional

or clear cell
malignant tumor made of transitional cells involving the collecting system
RCC
2/3 are men; >50 yo; only ~ 1% are bilateral

Bilaterally strongly suggestive of underlying syndrome
RCC
biggest risk factor for RCC?
SMOKING
von Hippel Lindau disease can be a risk factor for what?
RCC
von Hippel Lindau disease =
found in RCC
classic triad seen in RCC?
Flank pain

palpable mass

hematuria
Pt is in a car wreck and has suspected internal bleeding, upon imaging what tumor would likely be found?
RCC

most commonly found incidentally
25% of the time what is found at time of presentation for RCC?
metastases
5 year survival for RCC
70%
What can help determine overall 5 year survival? (2)
tumor stage and nuclear grade
How does chemo help in RCC?
it doesn't really
major tx of RCC?
surgical resection
genetics of RCC?
VHL gene (3p25) – mutation at this locus seen in >90% of cases
what does mutation of VHL do? in what cancer?
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
high levels of VFEG is seen in what?
clear RCC

because lack of VHL gene

will lead to a highly vascular tumor
what is TNM in tumor staging?
T – size of primary tumor; extent of invasion beyond kidney
N – presence of metastases to regional lymph nodes
M – distant metastasis
cyctoplasm clear with glycogen =
Clear cell RCC
germline mutation in c-met gene =
Papillary RCC
Associated with ESRD (“dialysis kidneys”)
Papillary RCC
More often multiple than any other type of RCC
Papillary RCC
size of a Papillary RCC
> 0.5 cm
which has a better prognosis Papillary RCC or clear?
Papillary RCC
Rare RCC with worst prognosis of subtypes. Typically presents as painless gross hematuria
Collecting Duct Carcinoma
A Mediterranean man presents with sickle-cell trait and a renal mass. What does he have
Renal Medullary Carcinoma

could be a black dude too
Neoplasms of transitional cell epithelium (TCC) =
Renal Urothelial Carcinomas
this is multifocal in development and undergoes the carcinogenic "feild effect" from tobacco
Renal Urothelial Carcinomas
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?
RCC
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?
VHL mutation