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29 Cards in this Set
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Young patient with proteinuria/hematuria/renal failure
Micro: Normal findings on H&E, minimal mesangial proliferation Stains: normal IF: negative EM: podocyte/ foot process effacement |
Minimal change disease
Can be idiopathic, or 2ndary to drugs/infections |
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Patient with hematuria/renal failure
Micro: Thickened mesangium, PAS highlights hypercellularity IF: IgA deposits in mesangium, some in capillary walls- granular deposits EM: dark-gray discoloration in thickened BM. Foot processes normal |
IgA nephropathy
If presents with abdominal pain & arthitis, then Henoch-Schoen purpura Idiopathic= Berger's disease Can be 2ndary to RA, celiac disease, liver disease, cancer |
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Patient with nephrotic syndrome
Micro: thickenend mesangium (<50%), PAS highlights thickened area without capillaries. Trichrome shows thickened area to stain blue. IF: nonspecific IgM, IgG deposits EM: Foot process effacement- they may shatter and be in urine stream. Podocytes detatching |
Focal Segmental Glumerulonephritis (FSGS)
Variants: Tip (reversible) Cellular Perihilar NOS Collapsing (2/2 HIV infection)- from podocyte proliferation Is Dx of exclusion- can be seen in other glomerulopathies |
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Patient with proteinuria/hematuria/renal failure
Micro: thickened looking mesangium- PAS highlights thickened capillary loops (thicker than capsule). Silver stain highlights thickened loops, shows "spiked" pattern IF: granular IgG deposits EM: shows dark gray deposits in BM. |
Membranous glomerulopathy
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Patient with nephrotic syndrome
Micro: focal areas of glomerular thickening. PAS highlights "nodular" sclerosis, mesangeal thickening, thickened capillary loops. Nodular areas stain with silver IF: IgG/ Albumin deposits EM: MArkedly thi9ckened capillary loops- 1.5-2 microns |
Diabetic nephropathy
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Young patient with nephrotic syndrome
Micro: thickened capillary loops and membranous proliferation on PAS. Silver stain shows doubling or "train track" appearance of BM. May see hyaline thrombi. IF: Granular IgG deposits EM: Thickened BM with dark-gray deposits, increased podocytes filling spaces (crowded) |
Membranous proliferative glomerulonephritis (MPGN)
Can be secondary to hepatits/malignancy, primary, or hereditary |
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Young patient with nephritic/nephrotic syndrome/renal failure
Micro: thickened mesangium with infiltrative pattern of neutrophils IF: Granular IgG/C3 deposits EM:Large "humps" of light gray material in urinary space |
Post-streptococcal glomerulopnephritis
Can be seen with strep, staph, viruses. If IgA dominant, can be MRSA |
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Most common cystic renal disease, and most common genetic disease (1:500), 4th MC cause of renal failure. Patients in 30s-40s. 25% lack family history
Genetics: AD, chr 16 and 4, mutations in polycystin 1/2 Gross: massive renal enlargement in adulthood with cysts that are sperical and unilocular. Non-uniform. The collecting system is normal Micro: single layer of epithelium, with focal polypoid areas. Fibrosis develops. |
Adult polycyctic kidney disease (AD-PKD)
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bilateral kidney disease AND liver cysts/fibrosis with dilitation of intrahepatic bile ducts (Caroli's disease).
Affects children, severe cases lead to neonatal death via pulmonary hypoplasia. Genetics: AR, PKHD1 on 6p12 Gross: massively inlarged kidneys, uniform, spngy appearance. Micro: elongated collecting duct cysts, normal nephrons. |
Autosomal recessive polycystic kidney disease (AR-PKD)
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Most common cause of unilateral renal enlargement in infants.
Genetics: complex, can be familial or sporadic, syndromic (LOTS) Gross: mutlicystic and anaplastic dysplasia.. many different appearances Micro: various based on etiology. Lobular areas of well-developed organization, alternate with large cysts. NOT organized normal structures. |
Renal dysplasia
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cystic renal disease causing failure not attributed to a hereditary cystic disease.
Typically bilateral cysts that grow over time. Accounts for 8% of dialysis patients. Present with flank pain and hematuria Gross: kidneys are NOT enlarged, typically normal or reduced in size. Cortical cysts common, later medullary. Micro: Cortex shows end-stage changes (glomerulosclerosis, tubular atrophy, interstitial fibrosis). Associated with RCC. |
Acquired renal cystic disease
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middle aged person, typically african american, with hypertension (essential), and slowly rising serum creatinine
Gross: kidneys are reduced in size, with granular cortical surface Micro: glomerulosclerosis, atrophic tubules, thickened arterioles. Collagenous tissue in Bowman's space and collapsed tufts (ischemic gloms). |
Benign nephrosclerosis
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middle aged person, typically african american man, with hypertension, renal failure, headache, dizziness, papilladema
Gross: Normal or large kidneys with petichiae or subcapsular hemorrhage Micro:fibrinoid necrosis, capillary loop thrombosis. Once treated, shows "onion skin" myointimal thickening |
Malignant nephrosclerosis
If unrevesable hypertension and kidney is small and smooth, think Renal artery stenosis |
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Patient with fever and flank pain, decreased urine, and elevated BUN and creatinine
Gross: enlarged, soft kidneys Micro: expansion of the interstitium by edema and variable inflammatory infiltrate- typically mononuclear with lymphocytes |
acute interstitial nephritis
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elderly man (2:1) with hematuria, occasionally flank pain and mass
Gross: mass in cortex, rarely bilateral, that is yellow (lipid) and hemorrhagic Micro: clear cells in alveolar pattern with delicate vessels. Variable anaplasia affects grading. Grade 2- nucleoli at >10x, grade 3 <10x IHC: RCC, EMA, CD 10, CD68, MUC1, MUC3 1. Incidence? 2. Where does it metastasize? |
Renal cell carcinoma (clear cell)
1. 2% of all malignancies, incidence increases with age. 2. Lung, bone, liver, adrenal Hereditary form: VHL genein 3p25 |
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elderly man (2:1) with hematuria, occasionally flank pain and mass
Gross: renal cortical mass with necrosis and hemorrhage, with fibrinous pseudocapsule. More commonly multifocal/bilateral Micro: true vascular cores with foamy macs surrounded by tumor cells. Psammoma bodies common. Two variants: type 1- single layer of low-grade tumor cells, type 2- abundant, eosinophilic cytoplasm (worse outcome) IHC: CK7 (type 1), CD9,10, EMA and vimentin are inconsistent |
Papillary RCC (if >5mm)
* if smaller, is renal papillary adenoma Genetics: chr 7, 17 trisomy and loss of Y. Hereditary form with mutations in the c-met gene |
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elderly man (2:1) with hematuria, occasionally flank pain and mass
Gross: spherical, well-circumscibed renal mass with pseudocapsule, homogeneous and tan. Micro:Round/polygonal tumor cells with thick vascular walls and clear borders. Finely granular eosinophilic cytoplasm that stains with Hale's colloidal iron stain... Eosinophilic variant with denser cytoplasm. Can undergo sarcomatoid transformation IHC: CK7 Genetics: lots of chromosomal losses, hereditary form (Brit-Hogg-Dube sndrome) |
Chromophobe RCC
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Rare tumor in younger patients, presents with hematuria. Erbb2 amplifications seen
Gross: large tumors originating in the medullary pyramid, although cortex is usually invovled. Micro: complex cords or tubular structures in a densely desmoplastic stroma Very poor prognosis |
Carcinoma of collecting duct of Bellini
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Young boy/adult with sickle cell trait with rapidly enlarging renal mass
Gross: solitary inflitrative mass in the medulla. extensive hemorrhage and necrosis. Micro: poorly differentiated neoplasm- solid sheets of cells with eosinophilic or amphophilic cytoplasm, puncuated with circular holes (lace-like). Embedded in a dense desmoplastic stroma IHC: CK, EMA, CEA |
Renal medullary carcinoma
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Young patient with hematuria and flank mass
Gross: yellow solitary cortical mass with hemorrhage and necrosis. Micro: papillary architecture with clear-cell features. IHC: TFE3 gene product |
XP11.2 translocation/TFE3 gene fusion-related renal carcinoma (MC is t(X,1)
variant t(X;17) ASP-TFE present at high stage and have alveolar architecture and psamomma bodies |
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Elderly man (2:1) with renal mass, usually asymptomatic but may have hematuria
Gross: solitary, well circumscribed, nonencapsulated with "mahogony brown" surface. A central scar occasionally seen. Micro: cells with abundant finely granular cytoplasm, growing in sheets or nests, with edematous hypocellular stroma. No mitoses, can have cytologic atypia. IHC: vimentin NEGATIVE. positive for most cytokeratins and EMA. Focal CK7. |
Oncocytoma
Color is from mitochondria, arise from intercalated cells of collecting ducts. |
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Polycythemia or asymptomatic renal mass in young woman
Gross: well-circumscribed, nonenapsulated mass with focal necrosis or hemorrhage Micro: densly packed small round cells in acini or tubules, focal papillary architecture. Psamomma bodies common. Cells are bland and uniform, oval, no mitoses. IHC: WT1, negative for CK, EMA, CK7 |
Metanephric adenoma
stromal tumor can be seen with similar features in neonates, CD34 positive |
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Young child (under 6) with palpable abdominal mass, elevated serum mucin and hyaluronic acid. 10% have dysmorphic syndrome, such as WAGR and del ch 11p13
Familial cases can be bilateral Gross: large, well circumscribed, solitary mass that distorts surrounding parenchyma. Can have cystic change Micro: 3 patterns: blastemal: small round cells with high N/C ratios, overlapping, mitoses epithelial: resembles normal renal morphology stromal:myxoid and immature spindle cells.. can have muscle or neuronal differentiation. typically a combination of all 3 types. Anaplasia predicts poor outcome IHC: WT1, vimentin |
Nephroblastoma/Wilms
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young boy (under 2) (2:1) with a renal mass.
Gross: unilateral irregularly shaped mass with cysts and light brown surface Micro:cords of cells with pale cytoplasm and pale vescicular chromatin, thin vessels separate with spindle cells. inflitrating. IHC: only vimentin positive. |
Clear cell sarcoma of the kidney
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young boy (under 2) with hematuriaand hypercalcemia, disseminated disease at presentation. Associated with PNET in the brain...
Gross: non-encapuslated mass that is infiltrative with pale cut surface and focal hemorrhage and necrosis Micro: sheets of large cells with vescicular chromatin, prominent nucleoli, and eosinophilic inclusions. LVS invasion common. IHC: vimentin, CK and EMA focally positive. |
Rhabdoid tumor of the kidney
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Woman of any age (3:1), can be sporadic or with tuberous sclerosis
Gross: well circumscribed, usually solitary lesion with yellow lobulated cut surface. Micro: combination of fat, blood vessels, and muscle. Can have atypia. Occasionally has large epithelioid cells with eosinophilic cytoplasm IHC: HMB-45/melan A, SMA, desmin, c-Kit |
Angiomyolipoma
Epithelioid variant can metastasize, kill: lack fat |
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young adult with pooly controlled hypertension, hypokalemia, renal mass, and elevated renin levels
Gross: small mass in renal cortex Micro: uniform polygonla cells with granular eosinophilic cytoplasm and distinct cell borders. In sheets or leaf like papillae. hemangiopericytic vascular arrangement. Cells show no atypia IHC: CD34, CD31, renin, vimentin, actin |
juxaglomerular cell tumor
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middle aged woman (8:1) with incidental renal mass
Gross: encapsulated mass of cysts, sharply demarcated from parenchyma. Micro: cysts are lined with flat cuboidal epithelial cells. The septa are densely fibrotic. IHC: ER/PR, inhibin. |
cystic nephroma
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Young patient with hematuria and flank pain (10% with mets)
Gross: very large (up to 16 cm) mass that replaces the kidney. Lobulated and white on cut surface, with necrosis and hemorrhage. Micro: small round blue cell tumor with frequent mitoses and pyknotic cells. pseudorosettes seen. IHC: CD99 (MIC2), HBA71, vimentin. 50% with FLI1 |
Primitive neuroectodermal tumor (PNET/ Ewing's)
Genetics: EWSR1-EWSR2 fusion t(11:22) |
