Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
48 Cards in this Set
- Front
- Back
|
Name the 5 benign renal tumors.
|
Oncocytoma
angiomyolipoma Adenoma Medullary fibroma Metastatic nephroma |
|
|
What are the 3 main malignant renal tumors?
|
Renal cell carcinoma
Wilm's tumor Transitional cell carcinoma |
|
|
What percent of transitional cell carcinomas occur proximally to the bladder?
|
5%
|
|
|
What percent of primary renal tumors are transitional cell carcinomas?
|
5-10%
|
|
|
What percent of renal neoplasias are oncocytomas?
|
5-10%
|
|
|
What is the gross appearance of a renal oncocytomas?
|
Well circumscribed mahogany brown lesion, mostly solitary.
|
|
|
What is the microscopic appearance of a renal oncocytomas?
|
monotonous bland eosinophillic cells packed with mitochondria with no nuclear atypia
|
|
|
What tissue types make up a renal angiomyolipoma?
|
Like the name suggests: fat, smooth muscle and vessels.
|
|
|
In what autosomal dominant condition do 25% of the patients have renal angiomyolipoma?
|
tuberous sclerosis (pts have hamartomas and benign brain/soft tissue tumors with skin nailbed, retinal, pulmonary and heart involvement)
|
|
|
What type of renal neoplasia is found in >40% of all autopsy pts over 70 y/o?
|
Renal adenomas
|
|
|
What is the gross appearance of a renal angiomyolipoma?
|
Encapsulated, yellow, and bosselated
|
|
|
What is the common size and microscopic appearance of a renal adenoma?
|
<5mm
Looks like a low grade papillary |
|
|
What renal neoplasia is found in 50% of all adult autopsies?
|
medullary fibroma (benign)
|
|
|
What's the gross appearance of a medullary fibroma?
|
<5mm well circumscribed pale gray lesion within the pyramid
|
|
|
What is the microscopic appearance of a medullary fibroma?
|
Fibroblast like cells with collagenous tissue
|
|
|
What is the most common pediatric renal neoplasia?
|
Mesoblastic nephroma
|
|
|
Who gets Mesoblastic nephromas?
|
Babies: 90% are less than 1 y/o, 63% are less than 3 months
|
|
|
What's the appearance of a Mesoblastic nephroma?
|
1-15 cm lesion of benign spindle cells of fibroblastic or myofibroblastic origin
|
|
|
What's important to keep in mind when resecting a Mesoblastic nephroma?
|
It can recur if the resection is incomplete
|
|
|
What is the most common solid abdominal tumor in kids?
|
Wilm's tumor (malignant neoplasm of embryologic nephrogenic elements)
|
|
|
What percent of Wilm's tumors are assoc with congenital syndromes?
|
5%
|
|
|
What congenital syndromes are assoc with Wilm's tumors?
|
WAGR
Denys-Drash Beckwith-Weidemann |
|
|
What is WAGR?
|
Congenital syndrome with Wilm's, Aniridia (no iris), GU anomalies, and retardation
|
|
|
What causes WAGR?
|
Chomosome 11 short arm deletion = affects PAX6 and WT1
|
|
|
What percent of WAGR pts get a Wilm's tumor? What has to occur for this to happen?
|
1/3
Must get mutation in the remaining WT1 |
|
|
What causes Deny-Drash syndrome and what do you see?
|
WT1 mutation = Wilm's, intersexual disorders, and glomerulonephropathy
|
|
|
What causes Beckwith-Weidemann syndrome and what do you see?
|
WT2 mutation = Wilms, gigantism, visceromegly, and macroglossia
|
|
|
What percent of familial Wilm's is bilateral?
|
20%
|
|
|
Who gets Wilm's and how does it present?
|
Young kids (2 y/o) with abdominal mass
|
|
|
What worsens the prognosis of a Wilm's tumor?
|
pt > 2 y/o, extension beyond the capsule, anaplasia
|
|
|
What is the prognosis of a Wilm's tumor?
|
90% long term survival with surgery, chemo and radiation
|
|
|
What makes up 80% of renal cancers?
|
Renal cell carcinoma
|
|
|
Who gets Renal cell carcinoma?
|
men older than 60
|
|
|
What's the classic triad of Renal cell carcinoma? What percent of patients actually get this?
|
Hematuria, flank pain, and abdominal mass. Only seen in <10%, most just have painless hematuria
|
|
|
What percent of Renal cell carcinoma are familial?
|
5% (Family Hx = 4-5x risk)
|
|
|
What's different about the familial Renal cell carcinomas?
|
They tend to be multifocal, bilateral, and occur at a younger age
|
|
|
What 3 conditions are associated with familial Renal cell carcinoma?
|
Von-Hippel Lindau
Autosomal Dominant RCC Hereditary papillary carcinoma |
|
|
What mutation is seen in 98% of the clear cell renal carcinomas?
|
VHL tumor suppressor gene
|
|
|
What chromosome is affected in autosomal dominant RCC and what percent develop cancer?
|
Chromosome 3
50% develop clear cell carcinoma |
|
|
What is the mutation and inheritance of Von-Hippel Lindau?
|
Autosomal dominant mutation in the VHL tumor suppressor gene
|
|
|
What neoplasias do you see in a patient with Von-Hippel Lindau? (4)
|
Cerebellar hemangioblastoma
Retinal Angioma Clear cell renal carcinoma (40%) Pheochromocytoma and cysts |
|
|
What is the abnormality in patients with hereditary papillary carcinoma of the kidney?
|
cmet mutation and trisomies of 7,16, or 17
|
|
|
What are the 4 types of renal cell carcinoma and what's the prevalence of each?
|
70-80% clear cell
10-15% papillary (type 1 = sm cell) 5% chromophobe 1% collecting duct |
|
|
Who gets the rare collecting duct varient of renal cell carcinoma?
|
Blacks with sickle cell disease
|
|
|
What are the 2 main risk factors for renal cell carcinoma?
|
Smoking
Cystic disease |
|
|
What percent of chronic dialysis patients end up developing renal cell carcinoma?
|
1-7%
|
|
|
What's the prognosis for renal cell carcinoma if encapsulated? What if it's met'd at dx?
|
90% 5 yr survival if it's within the capsule
30% if it has mets at dx |
|
|
Rank the types of renal cell carcinoma from best prognosis to worse.
|
Papillary type 1 and chromophobe
Papillary type 2 (more aggressive) Clear cell (50% 5 yr survival) Sarcomatoid (die w/in a year) |
