Y2S2B1 - Path - Renal

Front 1

Term for GFR ~50% of normal, serum BUN & creatinine WNL, pts asymptomatic

Back 1

Diminished Renal Reserve

Front 2

Term for GFR 20-50% of normal, pts have azotemia usually a/w anemia, HTN, polyuria, & nocturia

Back 2

Renal Insufficiency

Front 3

Term for GFR 5-20% of normal, pts have edema, metabolic acidosis, hypocalcemia, & may have other manifestations of uremia

Back 3

Chronic Renal Failure

Front 4

Term for GFR <5% of normal, terminal stage of uremia

Back 4

End-Stage Renal Disease

Front 5

in the GBM, ________ forms a network suprastructure to which other glycoproteins attach.

Back 5

Type IV collagen

Front 6

The overall (-/+) charge of the GBM is important in the virtually complete exclusion of albumin from the glomerular filtrate b/c albumin is a(n) (cationic/anionic) molecule.

Back 6

Negative; anionic

Front 7

Name the X-linked inherited disorder resulting in a deficiency of the enzyme alpha-galactosidase A. A deficiency of this enzyme results in the accumulation of ___________ in the glomeruli, which eventually leads to chronic renal failure.

Back 7

Fabry Disease; glycosphingolipids

Front 8

Which glomerular diseases tend to manifest usually as nephritic syndrome?

Back 8

Proliferative GN & Crescentic GN

Front 9

Which glomerular diseases tend to manifest usually as nephrotic syndrome?

Back 9

Minimal Change Dz (ALWAYS), Membranous GN, FSGS

Front 10

Which glomerular diseases manifest equally as nephritic/nephrotic?

Back 10

Mesangioproliferative GN & Membranoproliferative GN

Front 11

Mediators of Immune Glomerular Injury: a) _________ are a family of proinflammatory mediators derived from arachidonic acid. The b) ____________ not only lyses glomerular cells, but can also act as a mediator that activates mesangial cells, causing the mesangial cells to release injurious products such as oxidants and proteases. c)______________ injure glomerular cells via antibody-dependent cell-mediated cytotoxicity (ADCC) and possibly other mechanisms, without formation of immune complexes.

Back 11

a) Eicosanoids include: prostaglandins, thromboxanes and leukotrienes. b) membrane attack complex, C5b-9. c) Cytotoxic antibodies

Front 12

Once any renal disease reduces the GFR to __-__% of normal, progression to end-stage renal failure occurs at a relatively constant rate

Back 12

30 – 50%

Front 13

The prototypical exogenous pattern of Acute Proliferative GN is ____.
The prototypical endogenous pattern is _____.

Back 13

postinfectious glomerulonephritis; SLE

Front 14

Anti-streptococcal antibodies include:

Back 14

anti-streptolysin O (ASO) and anti-DNAse B

Front 15

electron dense subepithelial “hump” is characteristic of what?

Back 15

postinfectious/poststreptococcal GN

Front 16

Which type of RPGN is helped by plasmapheresis?

Back 16

Type I RPGN

Front 17

Which type of RPGN has ANCA? linear Ig & C3? immune complex, granular IG & C3?

Back 17

Type III, I, II. Tx consists of steroids, cytotoxic drugs, & plasmapheresis

Front 18

In RPGN, crescents are formed by proliferation of a)______ cells, infiltrates of b)________, & c)_______ deposition in Bowman’s space.

Back 18

a) parietal; b) WBC's; c) fibrin

Front 19

In RPGN, EM reveals ______ in the GBM.

Back 19

focal ruptures (this is a severe form of glomerular injury that allows RBC’s, fibrin, as well as other proteins, and inflammatory mediators to enter into the urinary space. the presence of fibrin and growth factors in the urinary space is then believed to stimulate the parietal epithelial cells to proliferate resulting in crescent formation)

Front 20

MC primary glomerular diseases causing nephrotic syndrome? 95% of children have primary and 60% of adults.

Back 20

minimal change disease (mostly children)
focal segmental glomerulosclerosis (all ages)
membranous glomerulopathy (mostly adults)

Front 21

MC systemic diseases causing nephrotic syndrome?

Back 21

diabetes mellitus, amyloidosis, SLE

Front 22

Some causes of secondary membranous glomerulopathy? Secondary is d/t immune complexes

Back 22

drugs (e.g., penicillamine, NSAIDs), malignancies (esp. carcinoma of lung & colon, melanoma), SLE, infections (e.g. hepatitis B & C, syphilis), Hashimoto thyroiditis

Front 23

diffuse thickening of glomerular capillary wall, subepithelial electron-dense deposits on EM, spikes w/ silver stain, granular Ig/complement on IF are a/w what?

Back 23

Membranous glomerulopathy. there is no increase in cellularity w/in the glomerulus

Front 24

In membranous glomerulopathy, what is a poor prognostic indicator IF it occurs?

Back 24

Glomerulosclerosis

Front 25

Proteinuria in Membranous Glomerulopathy is (selective/nonselective).

Back 25

nonselective & does not usually respond well to CS's, course is variable but indolent, only ~10% die or progress to CRF w/in 10 yrs

Front 26

The pathogenesis of primary membranous glomerulopathy involves:
a) An autoantibody directed against the GBM
b) An autoantibody directed against visceral epithelial cells
c) An autoantibody directed against mesangial cells
d) Deposition of circulating immune complexes

Back 26

B

Front 27

There is an increased incid of MCD (lipoid nephrosis) in pts with what?

Back 27

Hodgkin's dz (defects in CMI?)

Front 28

on EM of MCD, there is complete effacement of the foot processes, and the podocytes also exhibit ______ and _____.

Back 28

vacuoles and microvilli

Front 29

Some causes of secondary FSGS?

Back 29

renal ablation glomerulosclerosis, HIV infection & heroin addiction, secondary event in any focal glomerulonephritis

Front 30

T/F: Patients w/ FSGS often progress to chronic GN & develop end-stage
renal disease

Back 30

TRUE. FSGS --> eventually end up with diffuse GS --> ESRD --> dialysis

Front 31

segmental sclerosis of focal glomeruli, sclerotic segments have increased collagenous matrix & hyalinosis, IgM & C3; diffuse loss of foot processes w/ focal detachment of podocytes --> denudation of GBM

Back 31

FSGS. Hallmark is EPITHELIAL DAMAGE. you will see increased depositon of mesangial cells in only parts of some of the glomeruli. Hyalinosis is an area where plasma proteins have leaked through the glomerular capillary wall and have accumulated in the mesangium.

Front 32

Morphologic variant of FSGS typically seen w/ HIV infxn?

Back 32

Collapsing glomerulopathy

Front 33

some cases of FSGS are caused by mutations in genes that encode....?

Back 33

slit diaphragm proteins (e.g. nephrin, podocin & alpha-actinin 4). Podocin mutations may account for as many as 30% of cases of steroid-resistant nephrotic syndrome in children.

Front 34

In FSGS...a) ___% of patients progress to chronic GN
b) ___% of patients follow an unusally rapid course
c) recurrences are seen in ___% of patients receiving
allografts

Back 34

a) 50-80%. b) ~20%. c) 25-50%

Front 35

glomerular capillary wall has a “double-contour” or “tram-track” appearance caused by duplication of the GBM with interposition of mesangial cell processes; proliferation of mesangial cells with increased mesangial matrix and accentuation of the lobular architecture of the glomerulus

Back 35

MPGN
usually follows a slowly progressive relentless course, some patients develop RPGN, ~ 50% develop CRF within 10 years , high incidence of recurrence in transplant recipients

Front 36

subendothelial electron-dense deposits, granular deposits of C3 and often IgG, C1 & C4

Back 36

Type I MPGN

Front 37

lamina densa is extremely electron-dense (dense-deposit disease), C3 is present in the GBM & mesangium (mesangial rings), IgG, C1 & C4 are usually absent

Back 37

Type II MPGN

Front 38

Deposition of immune complexes with activation of both classical & alternative complement pathways is seen in which type of MPGN?

Back 38

Type I. Ag's involved are unknown

Front 39

low serum C3, factor B & properdin, but normal serum C1 & C4 (causes activation of alternative complement pathway) > 70% of patients have C3 nephritic factor (C3NeF), stabilizes alternative pathway C3 convertase. What is this?

Back 39

Type II MPGN

>70% of patients with dense-deposit disease have a circulating antibody termed C3 nephritic factor (C3NeF), which is an autoantibody that binds to the alternative pathway C3 convertase. Binding of the antibody stabilizes the convertase, protecting it from enzymatic degradation, resulting in persistent C3 activation. This causes the glomerular injury

Classical pathway C3 convertase cleaves C3 to C3a and C3b. C3b can then react with factors B and D from the alternative pathway to form alternative pathway C3 convertase, which also results in activation of the alternative pathway.

In the alternative pathway, C3 can be directly cleaved to C3a and C3b. The reaction depends on the interaction of C3 with substances such as bacterial polysaccharides, endotoxin and aggregates of IgA in the presence of factors B and D. C3b can then further react with alternative pathway C3 convertase to form alternative pathway C5 convertase (C3bBbC3b). Alternative pathway C5 convertase cleaves C5 to give rise to C5a and C5b. C5b can then react with complement factors 6 – 9 to form the membrane attack complex C5b-9. C3NeF binds to alternative pathway C3 convertase, stabilizing the convertase and protecting it from enzymatic degradation. This results in constitutive activation of alternative pathway C3 convertase, and in turn constitutive activation of the alternative pathway leading to glomerular injury.

Front 40

Some causes of secondary MPGN?

Back 40

chronic immune complex disorders, such as: SLE, hepatitis B & C, endocarditis, etc.
malignancies, such as: CLL, lymphoma & melanoma
hereditary deficiencies of complement regulatory proteins (such as I & H)

Front 41

MC GN type worldwide?

Back 41

IgA nephropathy aka Berger dzcourse
MC in children & young adults, present with hematuria often after a respiratory infection
hematuria tends to recur
slow progression to CRF in 15-40% of cases in 20 years
IgA deposits frequently recur in transplanted kidneys

Front 42

considerable variation on light microscopy: glomeruli may appear normal, may exhibit mesangioproliferative GN, focal proliferative GN or rarely crescentic GN
IF reveals mesangial deposition of IgA, often with C3 & properdin, early complement components are absent

Back 42

IgA nephropathy aka Berger dz
IgA1 & IgA1 immune complexes (d/t resp or GI Ag) are entrapped in the mesangium -> activation of alternative complement pathway -> glomerular injury

Front 43

Secondary causes of IgA nephropathy?

Back 43

liver disease
celiac disease
Henoch-Schönlein purpura

Front 44

areas exhibiting mesangial proliferation and increased deposition of mesangial matrix (this pattern is referred to as “mesangioproliferative glomerulonephritis)” is suggestive of (NOT diagnostic of) what?

Back 44

IgA nephropathy

Front 45

hereditary GN accompanied by nerve deafness, & various eye disorders. Males MC and more severe. Usually x-linked (sometimes AD or AR). presents as FSGS and the GBM is irregular & exhibits areas of thickening alternating with areas of thinning with lamination (splitting) of the lamina densa

Back 45

Alport Syndrome
hematuria, may have proteinuria, symptoms appear at 5 – 20 years of age, renal failure develops between 20 – 50 years of age (mostly in men)

Front 46

Nephritis in Alport is related to defective synthesis of the GBM. With X-linked disease, the defect is caused by mutations in the gene encoding what?

Back 46

alpha5 chain of collagen type IV
immunostains for the alpha5 chain performed on skin bx’s from patients with X-linked disease are negative. Mutations in the alpha3 & alpha4 chains are involved in the autosomal recessive and autosomal dominant forms.

Front 47

Thin Basement Membrane Lesion (Benign Familial Hematuria) is d/t mutations in genes encoding for...

Back 47

α3 or α4 chains of type IV collagen
Most patients are heterozygous for the defective gene & renal function is normal and prognosis is excellent. Homozygotes or compound heterozygotes, which are rare, may progress to chronic renal failure.

Front 48

T/F: some cases of chronic GN arise mysteriously with no antecedent Hx of any of the well-recognized forms of glomerulonephritis

Back 48

TRUE. kidneys are small with a thin cortex; there is hyaline obliteration (hyalinization) of most of the glomeruli. atherosclerosis & arteriolosclerosis is often prominent. secondary tubulointerstitial changes

Front 49

PKD1 gene on chromosome 16P encodes for polycystin 1, an integral membrane protein, and is defective in 85% of cases of what?

Back 49

Autosomal Dominant (Adult) Polycystic Kidney Disease.
PKD2 gene on chromosome 4q encodes for polycystin 2, an integral membrane protein (10-15% of cases). At least one additional gene is believed to be involved in a small % of cases

Front 50

AD hereditary disorder, always bilateral
accounts for 5-10% of chronic renal failure (CRF)
< 2% of affected individuals have CRF before age 40, 40% by age 60, & 75% by 70-75 years of age

Back 50

Autosomal Dominant (Adult) Polycystic Kidney Disease.

Front 51

Fluid moving through the renal tubules causes ciliary bending that opens _____ channels.

Back 51

Ca++. polycystin 1 & polycystin 2 form a complex in the cilia that regulates influx of Ca++ into the tubular epithelial cells in response to changes in fluid flow

Front 52

4-10% of patients with adult PKD die from what?

Back 52

ruptured berry aneurysms

Front 53

Autosomal Recessive (Childhood) Polycystic Kidney Disease involves what gene?

Back 53

PKHD1 on 6p21-23 encodes fibrocystin. fibrocystin is most likely a cell surface receptor with a role in collecting duct & biliary duct differentiation. Fibrocystin also has been localized to the cilia of renal tubular epithelial cells.

Front 54

The common feature b/w Nephronophthisis & Adult-Onset Medullary Cystic Disease is what?

Back 54

presence of cysts in the medulla and chronic tubulointerstitial nephritis

Front 55

Nephronophthisis, which affects children & young adults, has at least seven different genes identified. What are the 3 variants of it?

Back 55

sporadic, nonfamilial
familial juvenile nephronophthisis, AR
renal-retinal dysplasia, AR

Front 56

kidneys are small with a granular surface, small cysts in medulla, esp. at cortico-medullary junction, chronic tubulointerstitial nephritis
Patients present with tubular defects, e.g. polyuria, acidosis & Na+ wasting
Cysts may be too small to be visualized on imaging scans

Back 56

Nephronophthisis & Adult-Onset Medullary Cystic Disease

Front 57

Kidneys are enlarged, extremely irregular, w/ multiple cysts up to several cm in diameter On histo, there is disorganized architecture, with immature mesenchyme, cartilage, & immature collecting ducts (ex: a few cystically dilated ducts, as well as scattered islands of immature cartilage). This disorder is caused by an abnormality in what?

Back 57

abnormality in metanephric differentiation

may be unilateral or bilateral; most cases are a/w ureteropelvic obstruction, ureteral agenesis or atresia, & other anomalies of the lower urinary tract

Front 58

kidneys are mildly to moderately reduced in size; hyaline arteriolosclerosis and fibroelastic hyperplasia; patchy ischemic atrophy (foci of tubular atrophy & interstitial fibrosis, focal sclerotic glomeruli)

Back 58

Benign Nephrosclerosis
a/w “benign” hypertension
~ 5% eventually develop CRF (risk of CRF is increased in blacks, patients with more severe HTN, patients with both HTN & diabetes)

Front 59

What is the histologic finding that best correlates with the development of renal failure in patients with malignant hypertension?

Back 59

Hyperplastic arteriolosclerosis

Front 60

Patients w/ malignant nephrosclerosis often have an associated what?

Back 60

associated encephalopathy & retinopathy, may have scotomas
untreated malignant hypertension has a 90% mortality rate within a year

Front 61

How can you treat renal artery stenosis?

Back 61

Surgery cures up to 80% of patients if performed before hyaline arteriolosclerosis develops in the opposite kidney

Front 62

What is by far the most common type of fibromuscular dysplasia?

Back 62

The medial type

Front 63

In Hemolytic Uremic Syndrome (HUS), the _____ are the principal organ involved and _______ is the initiating event.

Back 63

Kidneys; endothelial injury

Front 64

In thrombotic thrombocytopenic purpura (TTP), the _____ is the principal organ involved, and ________ is the initiating event

Back 64

CNS (kidneys in ~50%); Platelet activation & aggregation

Front 65

Microangiopathic hemolytic anemia is characterized by what?

Back 65

the presence of fragmented RBC’s (schistocytes) in the peripheral blood smear.

Front 66

Typical (classic, epidemic) Hemolytic-Uremic Syndrome
cases occur after intestinal infection with what?

Back 66

strains of E. Coli that produce Shiga-like toxins (e.g. infected hamburgers)
MCly occurs in children w/ diarrhea, intestinal bleeding, oliguria, hematuria, MHA, thrombocytopenia , inc BP
most patients recover normal renal function in a matter of weeks, some patients may eventually develop CRF

Front 67

fibrinoid necrosis, thrombi in small vessels, cortical infarcts
hyperplastic arteriolosclerosis & cortical scars in chronic cases

Back 67

HUS/TTP

Front 68

Types of Atypical (non-epidemic) Hemolytic-Uremic Syndrome?

Back 68

Familial HUS (accounts for > 50% of cases of atypical HUS, patients have an inherited deficiency of a complement- regulatory protein, most patients have an inherited deficiency of Factor H. dec Factor H -> inc alternative pathway C3 convertase activity)
Antiphospholipid antibody syndrome
Scleroderma & malignant hypertension
Complication of pregnancy (postpartum renal failure)
Chemotherapeutic & immunosuppressive drugs

Front 69

MC in females <40 yo and is caused by an acquired or genetic deficiency of ADAMTS-13, a protease that cleaves very large vWF multimers

Back 69

Thrombotic Thrombocytopenic Purpura (TTP)

dec ADAMTS-13 activity -> accumulation of very large vWF multimers -> platelet aggregation -> microthrombi
majority patients with TTP have an autoantibody directed against ADAMTS-13

Front 70

How do you treat TTP?

Back 70

plasma exchange & immunosuppressive drugs

Front 71

platelet-fibrin thrombi within the glomerular capillaries is characteristic of what?

Back 71

a thrombotic microangiopathy

Front 72

What is the most common cause of acute renal failure?

Back 72

ATN (which is potentially reversible)

Front 73

Pancreatitis and peritonitis cause _________ of fluid within the abdomen, resulting in hypovolemia that reduces renal blood flow and can cause ATN.

Back 73

“third spacing”

Front 74

Heme containing proteins, such as myoglobin and hemoglobin are endogenous nephrotoxins. Other endogenous nephrotoxins include:

Back 74

uric acid and Bence-Jones proteins.

Front 75

Tubuloglomerular Feedback: inc delivery of _____ to the distal tubule -> inc release of _______ from the macula densa -> vasoconstriction of the afferent arteriole -> dec blood flow to the glomerulus -> dec GFR

Back 75

NaCl; adenosine

The sensor for this response is the macula densa. The amount of fluid entering the distal tubule at the end of the thick ascending limb of the loop of Henle depends on the amount of Na+ and Cl- in it. The Na+ and Cl- enter the macula densa cells via the Na+-K+-2Cl- cotransporter in their apical membranes. The increased Na+ causes increased Na+-K+ ATPase activity and the resultant increased ATP hydrolysis causes more adenosine to be formed. Presumably, adenosine is secreted from the basal membrane of the cells and acts via adenosine A1 receptors on the macula densa cells to increase their release of Ca2+ to the vascular smooth muscle in the afferent arteriole. This causes afferent vasoconstriction and a resultant decrease in GFR.

Front 76

In ATN d/t mercury poisoning, the _________ are spared.

Back 76

distal tubules

Front 77

Occasional patients with ATN do not have oliguria and may present with polyuria. These patients have mild renal tubular injury resulting in dec concentrating ability. This usually has a more benign clinical course and is seen most often with:

Back 77

exposure to nephrotoxins

Front 78

If the patient has normal concentrating ability, in the setting of (ATN/prerenal azotemia), the serum BUN/Cr ratio is typically > 20. In (ATN/prerenal azotemia), the BUN/Cr ratio is usually < 20.

Back 78

prerenal azotemia; ATN. The ratio of BUN to Cr can suggest hypovolemia, because of differences in the way each is handled in the nephron. Both substances are passively filtered at the glomerulus; however, whereas Cr remains within the tubule, urea is highly permeable to renal tubules and is passively reabsorbed with sodium. Therefore, in the setting of avid sodium retention, urea clearance is as low as 30 percent of GFR, whereas in the setting of adequate volume and sodium, urea clearance can increase to 70 to 100 percent of GFR.

Front 79

Pyuria & white blood cell casts in UA?

Back 79

Acute Tubulointerstitial Nephritis

Front 80

Tubulointerstitial suppurative inflammation, with abscesses & destruction of tubules; glomeruli are often spared. pregnancy & DM are predisposing factors. What are some possible complications of this?

Back 80

(this is acute pyelonephritis) papillary necrosis (diabetics, obstruction), pyonephrosis (obstruction), perinephric abscess

Front 81

MC form of chronic PN, starts in early childhood?

Back 81

Chronic Reflux-Associated PN (Reflux Nephropathy)

Front 82

coarse scars overlying blunted or deformed calyces, chronic interstitial inflammation & fibrosis, tubular atrophy & thyroidization, may have secondary glomerular changes

Back 82

Chronic pyelonephritis (an important cause of ESRD)

Front 83

The papillae at the poles are more concave, which allows for _______ to occur more readily at the poles.

Back 83

intrarenal reflux

Front 84

In chronic PN associated with ______, the scars occur predominately at the upper and lower poles where intrarenal reflux occurs more readily. In chronic PN associated with _________, scarring occurs more extensively throughout the kidney.

Back 84

reflux; obstruction

Front 85

Pt presents w/ fever, skin rash, eosinophilia, hematuria, proteinuria, sterile pyuria, axxotemia, increased serum IgE. Renal biopsy shows interstitial infiltration by mononuclear cells, eosinophils and neutrophils, “tubulitis”, may have interstitial granulomas. This can occur 2-40 days after the start of treatment by a variety of drugs such as:

Back 85

methicillin, ampicillin, rifampicin, thiazides, NSAIDs, etc.

drugs act as haptens and bind to tubular cells -> IgE and cell-mediated immune reactions

Front 86

Chronic tubulointerstitial nephritis with papillary necrosis caused by excessive intake of phenacetin-containing analgesic mixtures. Pts present with polyuria, nocturia, high BP, headache, GI symptoms, anemia, may develop UTI, may develop CRF. What can be useful in dx of papillary necrosis? What do these pts have an increased risk of?

Back 86

MRI & CAT scans
a/w increased risk of transitional cell carcinoma of the renal pelvis

Acetaminophen directly damages cells & aspirin promotes vasoconstriction -> ischemia

Front 87

Renal insufficiency occurs in ~___% of patients with multiple myeloma. What are the different ways it can cause it?

Back 87

50%. causes are
Bence Jones proteinuria & cast nephropathy (some light chains are directly toxic to tubular cells; Bence Jones proteins combine with Tamm-Horsfall protein to form tubular casts)
Amyloidosis
Light-chain deposition disease (light chains deposit in glomeruli -> glomerulopathy; light chains deposit in tubular basement membranes -> tubulointerstitial nephritis)
Hypercalcemia & hyperuricemia

Front 88

Inhibitors of crystal formation include:

Back 88

pyrophosphate, citrate, osteopontin & nephrocalcin

Front 89

T/F regard all papillary adenomas, regardless of size, as potentially malignant

Back 89

TRUE. do not differ histologically from low grade papillary renal cell carcinoma (RCC), have some cytogenetic features in common with papillary RCC, many have been identified in kidneys with RCC

Front 90

Accounts for ~ 70-80% of RCC’s, solid nests of tumor cells. In 98% of cases, there is loss of a VHL gene on chromosome ___. The other VHL gene exhibits mutations or is inactivated by hypermethylation

Back 90

Clear Cell CA. chromosome 3p

Front 91

Papillary CA accounts for 10-15% of RCC’s and is frequently multifocal in origin.
MC cytogenetic abnormalities are what?

Back 91

trisomies 7, 16, and 17 and loss of Y in male patients in sporadic cases, and trisomy 7 in familial cases
Gene for the familial form is the MET protooncogene on chromosome 7 (Met protooncogene encodes the receptor for hepatocyte growth factor)

Front 92

Accounts for 5% of RCC’s, sheets of cells with pale eosinophilic cytoplasm & perinuclear halos, arise from the intercalated cells of collecting ducts, cytogenetic studies reveal extreme hypodiploidy, have an excellent prognosis

Back 92

Chromophobe renal CA

Front 93

Accounts for ~ 1% of RCC, irregular channels lined by highly atypical epithelium surrounded by a fibrotic stroma, arise from collecting ducts, no specific pattern of cytogenetic abnormalities

Back 93

Collecting duct (Bellini duct) CA

Front 94

MC sites for metastases of RCC?

Back 94

Metastases are often the first manifestation of disease
MC sites for metastases: lungs, bone, regional nodes, liver

Overall 5-year survival rate is ~ 45%
Renal vein invasion or invasion through renal capsule into perinephric fat lowers 5-year survival rate to 15 – 20 %

Front 95

MC primary renal tumor in children (usually 2-5 yo), ~ 90% of cases are sporadic and unilateral. Triphasic pattern: blastema, stroma & epithelial elements. The tumor is soft in consistency, tan-gray in color, well-circumscribed, with scattered small cysts and focal mild hemorrhage. What makes a tumor less/more aggressive?

Back 95

Wilms Tumor (nephroblastoma)

Tumors composed predominately of epithelial elements are less aggressive. Tumors exhibiting anaplasia are more aggressive. they often have pulm metastases.

Front 96

What syndrome a/w increased risk of Wilms tumor has aniridia, genital anomalies & mental retardation, with a germline deletion at 11p13 --> loss of PAX6 & WT-1

Back 96

WAGR syndrome

mutation in the 2nd WT-1 allele -> Wilms tumor (1/3rd of children with WAGR syndrome develop Wilms tumor)
Deletion of the PAX6 gene results in aniridia. The WT1 gene encodes a transcription factor, which is critical for normal renal and gonadal development. WT1 gene is also a tumor suppressor gene.

Front 97

What syndrome a/w increased risk of Wilms tumor has gonadal dysgenesis, nephropathy, & germline missense mutation of WT-1.
~ 90% of these patients develop Wilms tumor

Back 97

Denys-Drash syndrome

Front 98

What syndrome a/w increased risk of Wilms tumor has organomegaly, hemihypertrophy, macroglossia and affected region is 11p15.5 (“WT-2”), but also overexpression of IGF2 gene is seen in many cases. There is an inc incidence of Wilms & other tumors

Back 98

Beckwith-Wiedemann syndrome

Front 99

painful chronic cystitis most commonly seen in women, present with severe suprapubic pain, frequency, dysuria and hematuria.
fissures seen on cystoscopy; ulcers with inflammation, granulation tissue and fibrosis
no bacterial infection; mast cells are prominent
? autoimmune origin

Back 99

Interstitial Cystitis (Hunner ulcers)

Front 100

A cystitis with inc incidence in immunosuppressed transplant patients, sheets of macrophages with abundant granular cytoplasm, macrophages contain phagosomes stuffed with bacterial debris
Michaelis-Gutmann bodies, related to E.coli & Proteus infections, most probably reflects an acquired defect in macrophage degradative function. grossly see focal areas with a slightly raised grey-tan plaque-like exudate

Back 100

Malacoplakia

Front 101

Painful, red 1- 2 cm nodular lesion, which occurs about the external urethral meatus in females, ulcerate and bleed easily, composed of inflamed highly vascular fibroblastic tissue, readily treated with surgical excision

Back 101

Urethral Caruncle

Front 102

Carcinomas arising in the posterior urethra are most commonly ___________.
Carcinomas arising in the anterior urethra are most commonly ___________.

Back 102

post - transitional cell CA, ant - squamous cell CA
Adenocarcinomas also arise within the urethra, but less frequently than transitional or squamous cell carcinomas

Front 103

Histo of urethra shows anastomosing cords of benign transitional epithelium, which appear to be growing down into the lamina propria. lack of any cytologic atypia, as well as the absence of any reactive stromal response. What is it?

Back 103

Inverted Papilloma