Renal Neoplasia - Pathoma Flashcards
What is an Angiomyolipoma?
Hamartoma composed of blood vessels, smooth muscle, and adipose tissue
- Angio = blood vessel
- myo = muscle
- lipo = fat (adipose tissue)
In what cases is there an increased frequency of Angiomyolipomas?
Tuberous sclerosis
What type of Renal neoplasia is a malignant epithelial tumor that arises from the kidney tubules?
Renal Cell Carcinoma
What is the classic presentation of Renal Cell Carcinoma?
Triad:
- Hematuria
- Palpable mass
- Flank pain Also common:
- fever
- weight loss
- paraneoplastic syndrome (EPO, renin, parathyroid horomone related peptide, ACTH)
How does a varicocele form in the case of Renal Cell Carcinoma?
Left-side only
- carcinoma obstructs gonadal vein draining into left renal vein
- blood backs up => varicocele Not on right side
- gonadal veing drains directly into inferior vena cava
Which type of renal neoplasm reveals a yellow mass on gross exam and clear cytoplasm microscopically?
Renal Cell Carcinoma(called clear cell type)
The pathogenesis of Renal Cell Carcinoma involves the loss of what gene?
VHL (3p) = tumor suppressor gene
What happens when you lose the expression of VHL in Renal Cell Carcinoma?
Increased IGF-3 (insulin-like growth factor)
- promotes growth Increased HIF transcription factor
- increases VEGF and PDGF (platelet-derived growth factor)
What are the two pathways in the development of Renal Cell Carcinoma?
Sporadic Pathway * single tumor in upper pole of kidney * usually in smoker Hereditary * tumors that arise in younger patients * multiple tumors and bilateral ***Both involve loss of VHL expression!
What is one autosomal dominant hereditary disorder that is associated with the inactivation of the VHL gene?
Von-Hippel Lindau Disease
Patients with Von Hippel-Lindau are at increased risk to develop what?
- Hemangioblastoma of the cerebellum
* Renal Cell Carcinoma
What is staging of Renal Cell Carcinoma based on?
T = based on tumor size and involvement of renal vein
- increases the risk of hematogenous spread to the lungs and bones
- N = spread to retroperitoneal lymph nodes
What do you call a malignant tumor of the kidney comprised of blastema, primitive glomeruli and tubules, and stromal cells?
Wilms Tumor
What is the most common malignant renal tumor in children?
Wilms Tumor(average age = 3 yoa)
What is the classic presentation of Wilms Tumor?
- Large, unilateral flank mass
- Hematuria HTN
- due to renin secretion