Tumors of kidney and urinary tract Flashcards
What are the 5 benign tumors mentioned in class?
Renal papillary adenoma (cortex) Renal fibroma or hamartoma (pyramids) Angiomyolipoma (pts. with tuberous sclerosis) Oncocytoma Metanephric adenoma
What are the 5 malignant tumors classified under Renal Cell Carcinoma (RCC)?
Clear cell carcinoma (VHL) Papillary carcinoma (Chromophilic) Chromophobe Renal Carcinoma Collecting Duct (Bellini Duct) Carcinoma Familial RCC
** can present with many paraneoplastic symptoms (EPO–>reactive polycythemia, Renin–>HTN, PTH–>hypercalcemia, ACTH–>cushing syndrome) [pathoma]
What are the clinical signs/symptoms and metastatic tendencies of RCC?
Triad: Hematuria, palpable mass, flank pain
Cortical renal mass
Metasteses often go to the heart (through renal vein), Lungs (multiple nodules)
Regional lymph nodes may be enlarged
Fever or weight loss can also present
What is the appearance of an RCC on gross pathological exam?
Single tumor (multifocal and bilateral in Von Hippel-Lindau disease) spherical YELLOW mass variegated appearance focal hemorrhage 20% are cystic.
What are the three cell types seen on light microscopic examination of an RCC biopsy?
Clear (most tumors are clear + granular)
Granular
Spindle (assx with more rapid/aggressive disease)
**Assign a nuclear grade based on Fuhrmans criteria (1-4). 1/2 have much better prognosis than 3/4.
Where is the VHL gene located? What is the function of this gene? What is the etiology of tumorgenesis in patients with this mutation?
VHL gene is located at 3p25.3. LOSS OF VHL in an unbalanced translocation or an outright deletion (3; 6, 8 or 11).
The VHL gene acts as a tumor suppressor in both sporadi and familial disease. VHL encodes part of a ubiquitin complex responsible for degradation, among other things, of Hif-1. When Hif-1 levels rise, it upregulates expression of VEGF, and TGF-B1 which are pro-angiogenic.
Insulin-like growth factor-1 is also upregulated, so both growth and angiogenesis are stimulated. PDGF also [pathoma].
What is the prognosis for clear-cell RCC?
45% after 5 years, 70% w/o distant metasteses.
What are the imaging/pathology findings for papillary carcinoma?
Frequently multifocal.
What cytogenetic abnormality is assx with papillary carcinoma?
Familial form: Trisomy 7. (MET locus)
Sporadic form often a trisomy of 7, 16, 17 or a loss of Y
**Better prognosis than ccRCC
Play the odds: what is the incidence of each of the 5 types of RCC?
ccRCC (70-80%) Papillary carcinoma (10-15%) Chromaphobe (1%) Collecting duct (Bellini) less than 1% Familial (4% of all renal cancers-1/2-2/3 are VHL patients)
What is the gross/histological appearance of chromaphobe RCC?
Cells with prominent cell membranes and pale eosinophilic cytoplasm, usually with a halo around the nucleus. Histologic distinction from oncocytoma can be
difficult.
**Excellent prognosis, similar to that of oncocytoma. Like oncocytoma, thought to grow from the intercalated cells of the CD
What is the genetic mutation INSIDE a chromaphobe RCC tumor?
extreme hyperdiploidy
Talk about Collecting Duct (Bellini) RCC
Pathology: Nests of malignant cells enmeshed within a prominent fibrotic stroma, typically in a medullary location.
Genetics: a distinct pattern has not been identified.
Prognosis: Associated with aggressive behavior and poor prognosis. For the majority of patients surgical treatment will not result in a cure.
How are renal tumors staged?
Using the TNM criteria. Different combinations of TNM values represent stages 1-4 (not outlined in our notes which combinations result in a staging classification. TNM is outlined in some detail in the notes.
T refers to the primary tumor and is assigned a number between (0-4; complex sub-stages)
N refers to the Regional lymph nodes and is assigned a number (0-2 ** 0 = none, 1 = one, 2 = more than 1)
M refers to the metasteses (0-1 scale; 0 = no distant metastasis, 1 = distant metastasis)
**In all categories, X means “unable to assess”
Describe the origin and frequency (general terms) urinary tract tumors.
Most tumors (95%) are of epithelial origin, called transitional or urothelial cell tumors. Squamous and glandular carcinomas also occur. Fibroepithelial polyps and leiomyomas also occur.
