Path - Renal Neoplasms Flashcards
What are the percentages associated with the 5 different categories of renal neoplasms
1) Clear Cell Carcinoma (70%)
2) papillary carcinoma (10%)
3) chromophobe carcinoma (5%)
4) oncocytoma (5%)
5) other - urothelial and squamous (10%)
Can a clear cell carcinoma of a small enough size be an adenoma (benign)?
No, all clear cell carcinomas are malignant
What is the exmple of an adenoma associated with the kidney ?
what percentage of population can you find them in?
are the completely benign?
small renal cortical papillary neoplasms (adenomas)
22% of population at autospy, these can be found
not entirely, reports of mets, “uncertain malignant potential”
none of this will probably be on the test anyway, but fuck it
Oncocytoma
1) benign or malignant
2) cell of origin
3) histo features
1) benign
2) renal tubular epithelial cells, the intercalated cells of the CD
3) large cells, many mitochondria, eosinophilic cytoplasm
what is the genetic signature of oncocytoma?
none
What is the gross appearance of oncocytoma
Mahogany Brown!!
can have a central stellate scar (30%) - good for the future radiologists?
oncocytoma - comment on the aggressive malignant potential of them
very very Rarely can be aggressive
they can get very large w/out being malignant - will not invade, will not metastasize
Histo features of oncocytoma
nuclei appear regular, maybe large ish, can see nucleoli (all nml)
cytoplasm - ton of mitochondria and eosinophilic color
for renal carcinomas in general
1) what is the appearance gross/ origin
2) what are 2 general pathologic process associated with them
1) large mass originating from tubular epithelial cells - look/ may be encapsulated
2) necrosis and hemorrhage
Why is necrosis common with renal carcinomas
renal vasculature comes from cortex, runs into the medulla - in general medulla is already hypoxic ish - so yeah, necrosis
why are renal carcinomas hemorrhagic
they are highly neovascularized to begin with, and they love to invade veins, and this adds to capacity to be hemorrhagic
What is the met pattern of renal carcinomas in general?
Hematogenous - go to lung, bone (brain, skin less common)
what are 3 features of the spread of generalized renal carcinomas
1) in general, where
2) another location
3) where do they rarely go
1) through capsule into perinephric fat
2) renal pelvis
3) rarely go to adjacent organs
Renal carcinomas love to invade veins - what is the process of this (where does it go, etc)
renal vein –> IVC –> R atrium
does not attach to the vessel wall, when you remove the kidney/tumor, can pull this tumor snake back out of the vessel
think about hemodynamic concerns associated with occluding your IVC like this
Renal carcinomas are hemorrhagic - when does this become a life threatening deal
when the thing mets to the brain, hemorrhage there, and then kick your bucket
what is the classic clinical triad for diagnosis of renal carcinomas
(High yield)
1) microscopic hematuria
2) dull flank pain, older male (very nonspecific)
3) abdominal mass (often times hard to find, ppl are fat)
1) are most renal carcinomas caught early or late?
2) and why
1) early
2) “excessive” CT scans / imaging
definition of clear cell carcinoma
malignant neoplasm composed of cells with clear or eosinophilic cytoplasm within a delicate vascular network
significance of delicate vascular network associated with clear cell carcinoma
VEGF
Buzz words for clear cell carcinoma
1) single or multiple
2) location
3) color
4) appearance
1) solitary
2) polar
3) yellow (not always though)
4) cystic
Genetics associated with clear cell carcinoma
chrom 3p abnormality
vast majority of these have something wrong with chrom 3p - whether it is sporadic or the inherited VHL type
What is the most important prognostic feature?
What is the next most important, and how do we measure this
clinical stage is most impt
nuclear grade is next most impt, Fuhrman
what are the general gross features of clear cell carcinoma
large, cystic, encapsulated, polar, variegated, hemorrhagic
we know what clear cell carcinoma looks like on histo
clear cells, noted vascularity, nuclei largely inconsequential
Fuhrman low nuclear grade
nuclei small, no mitotic figures, no nucleoli
Fuhrman high nuclear grade
nuclei variable size, irregular nucleol, also no mitotic figures
Fuhrman nuclear grading scale only applies to clear cell carcinoma
Genetic abnormalities associated with the sporadic type clear cell carcinoma
anything fucking with chrom 3
t(3;6), t(3;8), t(3;11)
deletion on chrom 3
genetic abnormalitites associated with hereditary form of clear cell carcinoma
VHL - which is on chrom 3
loss of VHL
inactivated or mutated VHL
hypermethylation of VHL
note: VHL mutation present in the McCoy family of the Hatfields and McCoys - played a role in why that feud got so out of hand (I mean, it really escalated quickly)
malignant, parenchymal tumor with papillary or tubulopapillary architecture
papillary carcinoma
papillary carcinomas more common solitary or multifocal?
unilateral or bilateral
multifocal and bilateral
what cytogenetic characteristics are associated with papillary carcinomas
none
Bob comes in to your office, has dull flank pain and an abdominal mass. you find out he is a chronic hemodialysis patient - what are you thinking?
acquired renal cystic disease from the hemodialysis leading to a 50% increase in risk for renal carcinoma (which for this vignette, we are assuming he now has; wrong side of coin flip for Bob)
60% clear cell
40% papillary carcinoma
(we talked about it with papillary, know that it is a significant percentage)
Bob died; here is his kidney
papillary carcinoma - can resemble clear cell carcinoma in many ways, grossly, maybe more cystic looking?
Here is th histo of Bob’s kidney
histo of papillary carcinoma
papillary structure, long, finger-like projections of tumor on a fibrovascular stalk
what is the genetics of papillary carcinoma
mutated activated Met - PRCC protoncogene
MET encodes a tyrosine kinase receptor of the hepatocyte growth factor
you take a histo section of some sort of renal tumor, stain it with Hale’s collodial iron stain and it lights up blue - what are you thinking?
Chromophobe carcinoma
What are the histo features of Chromophobe carcinoma
large pale cells, prominent cell membranes
will look worse than clear cell - looks more aggressive, big ugly nuclei (follow up question about this next)
what is the prognosis of chromophobe carcinoma
good
depsite the ugly aggressive looking nuclei, chromophobe is associated with a lower mortality
what is the cell of origin for a renal pelvis carcinoma
urothelial/transitional epithelial cells
tumors of the collecting system
what is an epidemiologic correlation for renal pelvis carcinomas
smokers
traditionally they get urothelial tumors of the bladder, over time they can spread to higher up in the collecting system
location of renal pelvis carcinoma
centrally, in the renal pelvis
(convenientally)
Bob’s wife Karen is giving their son, Billy, a bath when she notices an abdominal mass
Billy is somewhere between 2 and 5 years old
In your office, you note that billy has a fever and hematuria
what are you thinking
Wilm’s tumor / nephroblastoma
what percentage of kidney cancers in children does Wilm’s tumor comprise?
95%
what is the prognosis of Wilm’s tumor
good
post therapy 5 yr survival is 90%
microscopically, what is Wilm’s tumor composed of
mixture of cellular elements
blastema, stromal, and epithelial
Billy’s kidney - how does it appear grossly
Wilm’s tumor
big, encapsulated renal mass
looks a lot like any other kidney tumor, will need to use history and presentation to differentiate
what are the 3 syndromic associations for Wilm’s tumor (just the names)
1) WAGR
2) Denys-Drash
3) Beckwith-Wiedemann
aniridia (no irises), genital abnormalities and mental retardation
associated with a childhood kidney tumor
what syndrome is this?
WAGR
gonadal dysgenesis and renal abnormalities
associated with a childhood kidney tumor
what syndrome
Denys-Drash
enlargement of individual organs (organomegaly, often splenomegaly or hepatomegaly) and enlargement of one half of the body (hemimegaly, hemihypertrophy, hemihyperplasia)
associated with childhood kidney tumor
what syndrome is this
(probably high yield, Handorf liked this)
Beckwith-Wiedemann
all the hemi- stuff just means enlargement of the structures of one half of the body
How are you going to help Billy get better?
tx for Wilm’s tumor is nephrectomy and chemo
b/c it is a very aggressive neoplasm
Kidney is a highly vascularized structure that filters your whole blood volume a gazillion times a minute
so it is a very common site of metastasis, right?
what does a metastatic tumor of the kidney look like?
How do you remember the 4 disease manifestations of the WAGR syndrome
W - Wilm’s Tumor
A - aniridia
G- genital abnormalities
R -(mental) retardation
