Malignant stuff Flashcards
(32 cards)
Classic triad of RCC?
Is this common?
palpable mass
hematuria
flank pain
- uncommon; < 15%
The majority of RCC’s are _____, while the 3 most common subtypes are ___, ___, and ___.
Adenocarcinomas
Subtypes are:
- Clear cell (75%)
- Papillary (10-15%)
- Chromophobe (5%)
On average, what percentage of patients present with metastatic disease?
1/4 to 1/3 (25-33%
What are 3 histologic good prognostic signs?
- Increased CA-IX (carbonic anhydrase factor 9)
- low CA-IX expression is bad - Low vimentin
- vimentin is a mesenchymal intermediate filament; low expression means less differentiation/mutation - Low p53
- high p53 means more aggressive cancer
Any variant of RCC with ______ features has poor prognosis.
Sarcomatoid
All familial RCC disorders are autosomal recessive or dominant?
Dominant
What is the subtype of RCC that is most common in kids?
Papillary (still rare to see RCC in kids though)
What is the subtype of RCC that is most common in ESRD/dialysis (acquired cystic renal disease)?
How many years after being on dialysis is it recommended to start annual US screening?
Papillary RCC most common in ESRD/acquired cystic disease
Get screening US after 5 years of dialysis
A patient has a rapid development of right sided varicocele, what are you suspicious for?
Right sided RCC tumor venous invasion
When do you consider renal biopsy? Why?
Small renal masses < 4 cm
Why small?
- B/c 20% benign, 60% indolent malignancy, 20% aggressive malignancy.
- Low false negative rate (1%), low complication rate (<2%), and needle tract seeding is extremely rare (one case report)
Classically, what are the indications for renal biopsy?
Small renal masses < 4cm
Concern about metastasis
Abcess
Concern about lymphoma
What is the treatment for renal lymphoma?
Chemotherapy
What imaging workup should be pursued in patients with RCC?
CT scan: contrast enhancement >20 HU’s (i.e. no fat/AML)
MRI scan if renal vein or IVC involvement
CXR in all patients; CT chest if CXR inconclusive
CT brain if neurologic findings
Bone scan if elevated ALP, bone pain, or pathologic fracture
Mammogram in all women to r/o metastatic primary breast cancer
This is a subtype of RCC in which there is a purely cystic tumor lined by RCC clear cells (no solid component).
What is it associated with?
Treatment?
Multilocular Cystic RCC
Associated with VHL
Tx with surgical removal.
**no recurrence or metastasis reported after resection
Genetics of clear cell RCC?
mutated tumor suppression gene of VHL at 3p25-26
Clear cell RCC arises from which part of the nephron?
proximal tubules
What does the VHL gene normally suppress?
Hypoxia Inducible Factor-1 (HIF-1)
What is the normal physiological function of HIF-1?
under hypoxic situations, HIF-1 stimulates angiogenesis, glucose transport, and autocrine growth stimulation by activating the pathways of VEGF, PDGF, and Glut1
- therefore, if VHL is mutated, HIF-1 is intrinsically active and tumorigenesis proceeds
- these pathways are what the Tyrosine kinase, mTOR, and VEGF inhibitors target
Genetics of Papillary Type 1 RCC?
mutated proto-oncogene of cMet gene at 7q31
**this is the ONLY familial RCC proto-oncogene
What is the physiological function of cMet?
aka scatter factor, in regulates proliferation and differentiation of renal epithelial and endothelial cells
Papillary type 1 RCC arises from which part of the nephron?
proximal tubules
Histologically, papillary type 1 RCC appears?
Basophillic (blue) and low grade
Genetics of Papillary Type 2 RCC?
mutated tumor suppressor gene of fumarate hydratase at 1q42-44
*fumarate hydratase is a Krebs cycle enzyme, so if mutated, cells will continue to make ATP and grow
Papillary type 2 RCC arises from which part of the nephron?
Proximal tubules
