Renal Neoplasms Flashcards
Most common primary tumor of the kidney in children.
Wilm’s Tumor/ Nephroblastoma
Wilm’s tumor/Nephroblastoma is seen in kids what ages?
2-5 Toddlers
What are the 2 ways you can get Wilm’s tumor?
Sporadic - 90% of cases
Syndromic - associated with 3 syndromes..
Name the 3 syndromes associated with development of Wilm’s Tumor.
WAGR
Denys-Drash
Beckwith-Wiedemann
Abnormalities of what chromosome are associated with Wilm’s tumor in children?
Chromosome 11 - WT1 gene and WT2-gene cluster
specific WT2 gene has not been ID’d
Deletion of WT1 gene is associated with what?
WAGR syndrome
Mutations of WT1 gene….
Denys-Drash syndrome
Which syndrome is associated with increased size of body organs, especially tongue? What genetic abnormality causes Wilm’s tumor in this syndrome?
Beckwith-Wiedemann - Genomic imprinting disorder.
Overexpression of IGF-2 (insulin like growth factor 2) protein, resulting in organ enlargement and tumorigenesis.
What tumor is associated with loss of Chromosomes 1, 14, and Y chromosomes?
Oncocytomas
Are Oncocytomas benign or malignant?
Benign
what is the characteristic histology of an oncocytoma?
MITOCHONDRIA EVERYWHERE
On autopsy, what is the characteristic gross feature of oncocytomas?
Round, with a central stellate scar
Name the most common malignant renal neoplasm.
Renal cell carcinoma, specifically Clear Cell Carcinoma
Why are the cells clear?
Vacuoles filled with fat. Yellow color on gross observation.
Clear cell Carcinomas have what genetic abnormality?
2-hit mutations in the VHL tumor suppressor gene. Leads to uninhibited HIF (hypoxia inducible factor) activation of VEGF transcription.
What is the first symptom of Clear Cell Carcinoma? Why?
Painless Hematuria –> tons of weak, new vessels due to VEGF running rampant.
What’s the generic patient you’ll see with renal cell carcinomas? (specifically… what predisposes you to it..)
65 year old male SMOKER
How do Renal cell Carcinomas spread?
They spread equally between lymph and hematogenous. They like to invade the RENAL VEIN.
What are the 2 most common sites of metastasis of Renal Cell Carcinomas?
Lung - veins flow to lung
Bones (scapula) - nearest thing to it…
What is the second most common type of Renal Cell Carcinoma? How do you differentiate it from Clear Cell?
Papillary Renal Cell carcinoma.
Tend to be MULTIFOCAL and BILATERAL. Histology won’t show clear cells.
What mutation is associated with Papillary Renal Cell Carconimas?
MET proto-oncogene activating mutations.
MET is a tyrosine kinase receptor that’s activated by HGF (Hepatocyte growth factor)
“Have you MET PAP?”
What is the least common subset of Renal cell carcinoma?
Chromophobe Renal cell carcinoma.
What genetic abnormality is associated with Chromophobe Renal Cell Carconimas?
Total loss of multiple chromosomes. HYPODIPLOIDY.
What is the prognosis for each of the subsets of Renal Cell Carcinoma?
- Clear Cell - Bad
- Papillary - Bad
- Chromophobe - Good
