Path - Renal Neoplasms Flashcards by Abteen Moshref

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%)

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Can a clear cell carcinoma of a small enough size be an adenoma (benign)?

No, all clear cell carcinomas are malignant

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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

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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

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what is the genetic signature of oncocytoma?

none

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What is the gross appearance of oncocytoma

Mahogany Brown!!

can have a central stellate scar (30%) - good for the future radiologists?

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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

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Histo features of oncocytoma

nuclei appear regular, maybe large ish, can see nucleoli (all nml)

cytoplasm - ton of mitochondria and eosinophilic color

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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

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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

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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

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What is the met pattern of renal carcinomas in general?

Hematogenous - go to lung, bone (brain, skin less common)

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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

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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

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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

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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)

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1) are most renal carcinomas caught early or late?
2) and why

1) early
2) “excessive” CT scans / imaging

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definition of clear cell carcinoma

malignant neoplasm composed of cells with clear or eosinophilic cytoplasm within a delicate vascular network

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significance of delicate vascular network associated with clear cell carcinoma

VEGF

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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

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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

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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

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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