Path 63 Renal BV Disorders - Renal Cell Carcinoma

Can simple renal cysts become malignant?

NO!

Do renal cortical papillary adenomas present with clear cells? chromophobes?

NO!

Does FSGS occur in ADPKD?

NO!

what extra-renal disorders are associated with Alport's

Nerv deafness eye disorders because of similar type 4 collagen

50% of angiomyolipomas are associated with what condition?

tuberous sclerosis - flank pain, mass, hematuria, retroperitoneal hemorrhage

How does benign hypertensive nephrosclerosis affect the tubules and interstitium?

tubular atrophy interstitial fibrosis - picture shows thyroidization of the tubules which is a sign on chronic or end-stage kidney disease

Malignant hypertensive nephrosclerosis general age, ethnicity, and gender

young black males

Von Hippel-Lindau disease

• bilateral renal cell carcinomas • Hemangioblastomas (in cerebellum) • Pheochromocytoma (VHL gene)

Renal cell carcinoma decade of life and gender

6th decade of life male (2:1)

Malignant hypertensive nephrosclerosis blood pressure

>180 / >130 mmHg - note fibrin deposition.

List 6 extra-renal manifestations of ADPKD.

Berry aneurysm

What percent of end-stage renal disease are due to adult polycystic kidney disease?

4-6%

Does thrombotic thrombocytopenic purpura cause more severe renal or CNS disease?

CNS

Adult polycistic kidney disease gene affected

PKD1 gene

Childhood polycistic kidney disease gene

PKHD1 (6p21-23) - protein fibrocystin

List an autoimmune disorder, viral disease, and malignancy associated with thrombotic thrombocytopenic purpura.

SLE HIV hematological malignancy

what mutation and gene is associated with Renal cell carcinoma

VHL in chromosome 3 (Von Hippel Lindau) Loss of tumor suppressor VHL -> leads to proliferation of angiogenesis factors (PDGF, VEGF)

what is the most common solid tumor in children

Wilms tumor

which disorder has a "basket weave" appearance

alports

Describe the changes that occur to the arterioles and interstitium in ADPKD.

arteriolar sclerosis interstitial fibrosis - most likely due to compression (and hyper filtration?)

Adult polycystic kidney disease pattern of inheritance

autosomal dominant

Childhood polycistic kidney disease pattern of inheritance

autosomal recessive

List 3 risk factors for end-stage renal disease following benign hypertensive nephrosclerosis.

blacks (8-fold) higher blood pressure second underlying chronic disease (e.g. diabetes)

List other organs involved in malignant hypertensive nephrosclerosis.

brain eye heart lungs large vessels

what chromosome is associated with Wilms tumor

chromosome 11

Describe the changes that occur to the glomeruli, tubules, and interstitium in renal artery stenosis.(ipsilateral kidney)

crowded glomeruli atrophic tubules interstitial fibrosis (due to ischemia)

What epithelial lining makes up cysts in childhood polycystic kidney disease?

cuboidal - come from the collecting ducts (different from adult form)

What is the typical outcome for childhood polycistic kidney disease?

death in infancy or childhood - enlarged, cystic kidneys at birth

Do hepatic cysts in ADPKD affect liver function?

enlargement of the liver but focal to effected area. Therefore liver is functionally normal.

What pathogen is responsible for hemolytic uremic syndrome?

enterohemorrhagic E. coli (0157:H7)

What glomerular disorder can result from benign hypertensive nephrosclerosis due to nephron loss?

focal segmental glomerulosclerosis - compensatory hyperfiltration due to nephron loss

What is the most common sign of renal cell carcinoma?

hematuria - flank pain/palpable mass <10% of patients - *no RBC casts*

List 2 thrombotic microangiopathies.

hemolytic uremic syndrome thrombotic thrombocytopenic purpura

How does childhood polycistic kidney disease manifest in the liver?

hepatic fibrosis

What type of arteriosclerosis results from benign hypertensive neprhosclerosis of small blood arteries?

hyaline arteriosclerosis - due to extravasation of plasma proteins through injured endothelium

What happens to the contralateral kidney in renal artery stenosis?

hypertensive arteriosclerosis due to increased systemic pressure - arterioles in ipsilateral kidney protected from transmission of high pressure due to the stenosis --> no significant arteriosclerosis

List 3 organs that undergo ischemic injury due to microvascular thrombi in thrombotic microangiopathies.

kidney brain heart

How can benign hypertensive nephrosclerosis manifest in the heart, eyes, and brain?

left ventricular failure retinopathy stroke

What type of nephrosclerosis is associated with an onion-skin appearance?

malignant hypertensive nephrosclerosis

What 2 layers of the medium sized arteries are thickened in benign hypertensive nephrosclerosis?

media intima

What 3 structures make up the triphasic pattern of angiomyolipomas?

muscle fat vessels

Renal cell carcinoma tx

nephrectomy (radical or partial) "yellow sphere at one pole"

micro slide of Wilms tumor

note triphasic appearance

What are the 3 most common cause of renal artery stenosis?

occlusion by atheromatous plaque (75-90%) fibromuscular dysplasia (10-25%) - more common in female others: Takayasu's arteritis, aortic/renal artery dissection

Benign renal tumor that has a mahogany color with a light-colored area of scarring (gross description)

oncocytoma - oncocytes have homogeneous eosinophilic cytoplasms (rich in mitochondria)

List 2 endocrine disorders that can cause malignant hypertensive nephrosclerosis.

pheochromocytoma primary hyperaldosteronism

Does hemolytic uremic syndrome cause more severe renal or CNS disease?

renal

Renal cell carcinoma investigations (4)

renal US CT scan IV pyelograph biopsy - urine cytology is not good because architecture is more important for dx

Auscultation reveals a flank or epigastric bruit.

renal artery stenosis

Malignant tumor of renal tubular epithelial cells

renal cell carcinoma

Most common cancer of the kidney

renal cell carcinoma (90%)

Is renal cell carcinoma usually sporadic or inherited? unilateral or bilateral? single or multiple?

sporadic unilateral single These clear cells are indications of RCC

Disorders characterized by abnormal platelet aggregation leading to thrombosis in arterioles and capillaries throughout the body

thrombotic microangiopathies - schistocytes

what tissue pattern is Wilms tumor

triphasic