Kidney Malignancies Flashcards
(35 cards)
Most common renal cancer in adults? Where is it located?
Renal cell carcinoma.
This neoplasm originates in the cortex.
Where are “urothelial neoplasms”?
In the epithelium of the renal pelvis and below.
Most common renal cancer in children?
Nephroblastoma (Wilm’s tumor)
Major risk factors for renal cell carcinoma?
Tobacco use
Heavy metal and petroleum exposure
HTN, obesity, estrogen, etc.
(majority are sporadic, not linked to genetics)
Typical presentation of renal cell carcinoma?
What’s the classic triad of signs/symptoms?
Generalized symptoms: fever, weight loss, malaise.
Classic triad: hematuria, flank pain, and renal mass.
(hematuria is most sensitive, but only 10% of pts have all 3. Of people who have all 3, 50% already have mets)
How can renal cell carcinoma mimic many other diseases?
A lot of paraneoplastic processes can occur... E.g.: Polycythemia via EPO production. Anemia. Hypercalcemia via PTH-related protein. Hypertension via renin.
3 most common histologic variants of renal cell carcinoma (RCC)?
Clear cell - 70% of all RCC.
Papillary.
Chromophobe.
With what heritable syndrome is clear cell RCC associated? (sporadic clear cell RCC pts have mutations in the same gene - it takes 2 hits to get RCC)
Von Hippel Lindau syndrome - mutation in VHL.
Must RCC be large in order to metastasize?
No. It can metastasize when <1cm, but this is rare.
Gross appearance of RCC? (how does this correspond to histological appearance)
Pseudocapsule
Yellow (lots of lipid… which is washed out during fixation, making the cells look “clear”)
Hemorrhage and necrosis
How vascular are RCC tumors?
Very vascular. You can see capillaries running throughout them on histology.
What is grading of clear cell RCC based upon? (Fuhrman grading)
Nucleus morphology - small, dense like a lymphocyte is good.
Large, open chromatin with nucleoli is bad.
What factor does VHL suppress?
HIF, under normoxic conditions.
If VHL isn’t present HIF activity -> very vascular tumors.
4 examples of genes turned on by HIF that promote tumorigenesis?
VEGF
PDGF
TGF-alpha
GLUT-1 (insulin-independent glucose transporter)
Most frequent site of metastasis from RCC?
Lungs, then bone - but they can go “unusual” places.
it can travel along renal vein -> vena cava -> heart -> lung
Clear cell RCC responds poorly to chemotherapy.. what are 2 interesting more specific therapies being investigated for RCC?
VEGF inhibitors.
mTOR inhibitors
Where do nephroblastomas come from?
They’re embryological remnants - “nephrogenic blastema cells”
Does nephroblastoma have an association with any genetic syndromes?
Yes. 10% of nephroblastomas are associated with dysmorphic syndromes (WAGR, Beckwith-Wideman, Denys-Drash… don’t worry about knowing these)
Clinical presentation of nephroblastoma?
Abdominal mass (commonly incidentally discovered by parent). Abdominal pain, hematuria, HTN, and acute abdominal crisis (due to trauma). Rarely, kidney-related paraneoplastic stuff: anemia, HTN (from renin), polycythemia (from EPO)
Gross appearance of nephroblastoma?
Sharply-deliniated.
Tan, commonly with hemorrhage and necrosis.
(these can get quite big - photo showed one dwarfing the remnant normal kidney)
What the characteristic histology of nephroblastoma?
Renal tumor with 3 components:
Epithelium, blastema, and stroma.
What constitutes unfavorable histology in nephroblastoma?
Anaplasia (ugly cells with ugly nuclei)
Prognosis for nephroblastoma?
Very good (as far as these things go…)- 90% survival at 2 years, and 2 year survival usually means cure.
What gene is responsible for nephroblastoma/ Wilm’s tumor?
WT-1, a tumor supressor.
