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AP reviewer > Kidney > Flashcards

Kidney Flashcards

1
Q

Infantile polycystic kidney disease: Inheritance.

A

Autosomal recessive.

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2
Q

Infantile polycystic kidney disease:

A. Gene and its location.
B. Encoded protein.

A

A. PKHD1 on chromosome 6p21-23.

B. Polyductin / fibrocystin.

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3
Q

Infantile polycystic kidney disease: Presentation (2).

A

Abdominal distention.

Stillbirth or early neonatal death in most cases.

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4
Q

Infantile polycystic kidney disease: Associated abnormalities (2).

A

Pulmonary hypoplasia.

Congenital hepatic fibrosis.

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5
Q

Infantile polycystic kidney disease: Gross pathology.

A

Massive enlarged kidneys with smooth surface.

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6
Q

Infantile polycystic kidney disease:

A. Location and arrangement of cysts.
B. Calyceal system.

A

A. Cortex and medulla; oriented perpendicular to the renal capsule.

B. Normal.

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7
Q

Infantile polycystic kidney disease: Histopathology (2).

A

Dilated collecting ducts lined by bland cuboidal cells.

Nephrons appear normal.

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8
Q

Medullary cystic disease: Presentation.

A

Renal dysfunction in childhood:

− Polyuria and polydipsia.
− Uremia.
− Growth retardation.

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9
Q

Medullary cystic disease: Gross pathology.

A

Kidneys are small; both are affected.

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10
Q

Medullary cystic disease: Number, size, and location of cysts.

A

Many.

Up to 2 cm in diameter.

Corticomedullary junction.

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11
Q

Medullary sponge kidney: Presentation.

A

Found in children or adolescents.

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12
Q

Medullary sponge kidney: Gross pathology.

A

Kidneys are typically of normal size.

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13
Q

Medullary sponge kidney: Size and location of cysts.

A

Less than 0.5 cm.

Medullary pyramids, renal papillae; communication with collecting ducts.

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14
Q

Medullary sponge kidney: Prognosis.

A

Renal function is usually normal.

Progression to end-stage renal disease occurs rarely.

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15
Q

Renal dysplasia: Clinical significance (2).

A

Dysplastic kidneys usually do not function.

Nearly always accompanied by other abnormalities of the urinary tract.

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16
Q

Renal dysplasia: Presentation (2).

A

If large: Palpable mass.

If small: May remain asymptomatic for many years.

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17
Q

Renal dysplasia: Possible outcomes in utero (2).

A

Oligohydramnios with Potter’s sequence.

Death from pulmonary hypoplasia.

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18
Q

Renal dysplasia: Gross pathology.

A

Distorted kidneys with variable of cysts.

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19
Q

Adult polycystic kidney disease: Type of inheritance.

A

Autosomal dominant.

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20
Q

Adult polycystic kidney disease: Genes, locations, proteins (2).

A

PKD1, chromosome 16, polycystin 1.

PKD2, chromosome 4, polycystin 2.

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21
Q

Presentation of adult polycystic kidney disease:

A. Age of patient.
B. Laboratory abnormalities (2).

A

A. Fourth or fifth decade.

B. Hematuria or proteinuria.

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22
Q

Adult polycystic kidney disease: Extrarenal manifestations (4).

A

Berry aneurysms.

Colonic diverticula.

Cysts in pancreas, liver.

Cardiac valvular abnormalities.

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23
Q

Adult polycystic kidney disease: Gross pathology (2).

A

Early: Normal-sized kidneys with a few cysts.

Later: Markedly enlarged kidneys with irregular contour due to cysts.

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24
Q

Adult polycystic kidney disease: Size and location of cysts.

A

From a few millimeters to several centimeters in diameter.

Cortical and medullary.

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25
Q

Adult polycystic kidney disease: Histopathology (2).

A

Cysts: Flat or cuboidal epithelial lining.

Parenchyma: Fibrosis, atrophy.

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26
Q

Adult polycystic kidney disease: Complications (2).

A

End-stage renal disease.

Renal-cell carcinoma.

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27
Q

Acquired cystic disease: Presentation (2).

A

Usually asymptomatic.

Cysts develop in the setting of chronic renal failure, usually dialysis dependent.

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28
Q

Acquired cystic disease:

A. Inheritance.
B. Extrarenal manifestations.

A

A,B. None.

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29
Q

Acquired cystic disease:

A. Gross pathology.
B. Size and location of cysts.

A

A. Kidneys are moderately enlarged at the most.

B. Variable as in ADPKD; cortical.

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30
Q

Acquired cystic disease: Parenchymal changes.

A

Fibrosis, atrophy.

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31
Q

Acquired cystic disease: Complication.

A

Renal-cell carcinoma (can arise in a cyst or in the parenchyma).

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32
Q

Xanthogranulomatous pyelonephritis: Clinical significance.

A

Can form mass(es) and mimic renal neoplasia.

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33
Q

Xanthogranulomatous pyelonephritis: Most common agents (2).

A

Proteus spp.

E. coli.

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34
Q

Xanthogranulomatous pyelonephritis: Associated renal disease.

A

Calculi.

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35
Q

Xanthogranulomatous pyelonephritis: Progression.

A

Begins in renal pyramids and spreads to renal pelvis and then to capsule and to perinephric fat.

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36
Q

Xanthogranulomatous pyelonephritis: Gross-pathologic types (2).

A

Diffuse: More common.

Segmental: Polar; occurs in children.

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37
Q

Xanthogranulomatous pyelonephritis: Histopathologic zones of a nodule.

A

Inner: Necroinflammatory debris that is rich in neutrophils.

Outer: Lipid-laden macrophages.

Outermost (sometimes): Multinucleate giant cells, spindled fibroblasts.

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38
Q

Xanthogranulomatous pyelonephritis: Histopathologic pitfalls (2).

A

Lipid-laden macrophages may be confused with RCC.

Multinucleate giant cells and spindled fibroblasts may be confused with sarcomatoid carcinoma.

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39
Q

Angiomyolipoma: Inheritance.

A

Most tumors are sporadic.

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40
Q

Angiomyolipoma: Relation to tuberous sclerosis (2).

A

20% of tumors as associated with TS.

Most patients with TS develop an angiomyolipoma.

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41
Q

Angiomyolipoma: Relation of inheritance to gross pathology (2).

A

Sporadic: Large, single, unilateral.

TS: Smaller, multiple, bilateral.

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42
Q

Angiomyolipoma: Components.

A

Thick-walled hyalinized blood cells.

Smooth muscle.

Mature fat.

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43
Q

Angiomyolipoma: Possible atypical gross findings (2).

A

Presence of tumor in renal vein or in regional lymph nodes.

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44
Q

Angiomyolipoma: Possible atypical histopathologic features (3).

A

Nuclear pleomorphism.

Mitotic activity.

Epithelioid smooth-muscle cells.

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45
Q

Angiomyolipoma: Immunohistochemistry (2,1).

A

Positive: Melanocytic markers, smooth-muscle markers.

Negative: Epithelial markers.

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46
Q

Angiomyolipoma: Importance of careful sampling.

A

To exclude a coexisting renal-cell carcinoma.

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47
Q

Epithelioid angiomyolipoma:

A. Behavior.
B. Presentation.

A

A. Potentially malignant but unlikely to metastasize.

B. Flank pain or no symptoms.

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48
Q

Epithelioid angiomyolipoma: Radiography.

A

Lack of fat imparts resemblance to RCC.

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49
Q

Epithelioid angiomyolipoma: Association.

A

Tuberous sclerosis.

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50
Q

Epithelioid angiomyolipoma: Gross pathology (3).

A

Solid, hemorrhagic cut surface.

More likely than conventional angiomyolipoma to be necrotic.

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51
Q

Epithelioid angiomyolipoma: Cellular components (3).

A

Short spindle cells.

Large epithelioid cells.

Pleomorphic cells that resemble ganglion cells or multinucleate giant cells.

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52
Q

Epithelioid angiomyolipoma: Immunohistochemistry.

A

Similar to that of conventional angiomyolipoma.

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53
Q

Epithelioid angiomyolipoma: Significance of invasion of renal vein.

A

Observed more frequently in clinically malignant cases.

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54
Q

Epithelioid angiomyolipoma: Sites of metastasis (4).

A

Lymph nodes.

Lungs.

Liver.

Mesentery.

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55
Q

Metanephric adenoma:

A. Age group.
B. Possible “paraneoplastic” finding.

A

A. Most common in the fifth decade.

B. Polycythemia.

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56
Q

Metanephric adenoma: Cytology.

A

Small, bland cells with dark nuclei and scant cytoplasm.

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57
Q

Metanephric adenoma: Histologic architecture (3).

A

Small, tightly packed acini in an acellular stroma.

Variations:
− Papillary foci.
− Solid (blastema-like) foci.

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58
Q

Metanephric adenoma: Immunohistochemistry (3,2).

A

Positive: Pancytokeratin, CD57, WT-1.

Negative: CK7, AMACR, CD56.

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59
Q

Metanephric adenoma: Electron microscopy (2).

A

Basal lamina, microvilli.

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60
Q

Metanephric adenoma: Mutation.

A

V600E in BRAF.

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61
Q

Metanephric adenoma: Behavior.

A

Benign.

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62
Q

The nuclear grading system of Fuhrman.

A

1: 20 μm, pleomorphic, open or dark chromatin, macronucleolus.

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63
Q

Papillary adenoma: Associations (3).

A

Long-term hemodialysis.

Chronic pyelonephritis.

von Hippel−Lindau syndrome.

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64
Q

Papillary adenoma:

A. Size.
B. Histologic architectural patterns (3).

A

A. Less than 5 mm.

B. Tubular, papillary, or both.

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65
Q

Papillary adenoma: Cytology (2).

A

Bland cuboidal cells.

Nuclei correspond with Fuhrman grades 1 and 2.

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66
Q

Papillary adenoma: Other histologic features (2).

A

Psammomatous calcifications.

Foamy histiocytes.

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67
Q

Papillary adenoma: Disqualifying cytologic feature.

A

Clear cytoplasm.

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68
Q

Papillary adenoma: Immunohistochemistry (2,1).

A

Positive: CK7, AMACR.

Negative: WT-1.

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69
Q

Papillary adenoma: Mutations (3).

A

+7, +17, -Y.

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70
Q

Renal oncocytoma: Radiography.

A

Central scar (“spoke-wheel” appearance).

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71
Q

Renal oncocytoma: Histologic patterns (3).

A

Classic: Well-defined nests of oncocytes.

Tubulocystic: Tubules and cysts contain eosinophilic secretions.

Mixed: Nests and tubules.

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72
Q

Renal oncocytoma: “Tolerable” histopathologic anomalies (3).

A

Occasional smudged nuclei.

Occasional clear cells.

Focal extension into perinephric fat.

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73
Q

Renal oncocytoma: Features that are incompatible with the diagnosis (4).

A

Gross extension into the perinephric fat.

Papillae.

Sarcomatoid or spindle-cell areas.

Atypical mitotic figures.

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74
Q

Renal oncocytoma: Immunohistochemistry (4,1),

A

Positive: CK7, S100, E-cadherin, claudin 8 (cytoplasmic).

Negative: Vimentin.

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75
Q

Renal oncocytoma: Special stain.

A

Negative: Hale’s colloidal iron.

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76
Q

Renal oncocytoma: Mutation.

A

t(5;11) in some cases.

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77
Q

Renal oncocytoma vs. clear-cell RCC with eosinophilic cytoplasm: Immunohistochemistry (3).

A

Clear-cell RCC expresses vimentin but neither CK7 nor E-cadherin.

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78
Q

Renal oncocytosis: Causes (2).

A

Sporadic.

Chronic renal failure.

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79
Q

Renal oncocytoma: Putative origin.

A

Intercalated cells of the collecting ducts.

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80
Q

Birt-Hogg-Dubé syndrome: Renal tumor.

A

Combines features of renal oncocytoma and chromophobe RCC.

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81
Q

Renal-cell carcinoma, clear-cell type: Classic presentation.

A

Pain, flank mass, hematuria: Reported in only 10% of patients.

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82
Q

Renal-cell carcinoma, clear-cell type: Genes (4).

A

VHL.

PBRM1.

BAP1.

SETD2.

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83
Q

Renal-cell carcinoma, clear-cell type vs. clear-cell papillary renal-cell carcinoma: Immunohistochemistry.

A

Clear-cell papillary renal-cell carcinoma . . .

− Positive: CK7 and keratin 34βE12.
− Negative: CD10, AMACR.

Both tumors express CA9.

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84
Q

Renal-cell carcinoma, clear-cell type vs. adrenocortical carcinoma: Immunohistochemistry (2).

A

Adrenocortical carcinoma . . .

− Positive: Inhibin, calretinin.
− Negative: EMA, cytokeratins.

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85
Q

Clear-cell papillary renal-cell carcinoma: Cytology (2).

A

Clear cells with low-grade nuclei.

Nuclei are oriented away from the basement membrane and toward the lumen.

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86
Q

Renal-cell carcinoma, papillary type: Syndrome, gene, location.

A

Hereditary papillary RCC syndrome: c-met on chromosome 7q31.

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87
Q

Renal-cell carcinoma, papillary type: Gross pathology (2).

A

Fibrous pseudocapsule.

May be multifocal or bilateral.

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88
Q

Renal-cell carcinoma, papillary type, type I: Histopathology.

A

Papillae are lined by a single layer of cells with low-grade nuclear features and scant cytoplasm.

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89
Q

Renal-cell carcinoma, papillary type, type II: Histopathology.

A

Papillae are lined by pseudostratified cells of higher nuclear grade and much cytoplasm.

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90
Q

Renal-cell carcinoma, papillary type: Cytokeratins.

A

CK7 is expressed by 80% of tumors of type I and by 20% of tumors of type II.

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91
Q

Renal-cell carcinoma, papillary type: Additional immunohistochemical stains (3).

A

Positive: RCC antigen, CD10, PAX8.

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92
Q

Renal-cell carcinoma, papillary type: Mutations (3).

A

+7, +17, -Y.

No mutation of VHL.

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93
Q

Renal-cell carcinoma, chromophobe type: Syndrome.

A

Birt-Hogg-Dubé syndrome.

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94
Q

Renal-cell carcinoma, chromophobe type: Histopathology.

A

Thick cell membranes resembling walls of plant cells.

Koilocyte-like wrinkled nuclei with halo.

Thick-walled, hyalinized blood vessels.

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95
Q

Renal-cell carcinoma, chromophobe type: Histopathologic types.

A

Classic: Pale cytoplasm.

Eosinophilic: Intensely eosinophilic cytoplasm.

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96
Q

Renal-cell carcinoma, chromophobe type: Electron microscopy (2),

A

Classic: Many microvesicles.

Eosinophilic: Many mitochondria.

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97
Q

Renal-cell carcinoma, chromophobe type vs. oncocytoma: Stains (3).

A

Renal-cell carcinoma, chromophobe type . . .

− CK7, Hale’s colloidal iron: Diffusely positive.
− S100A1: Not expressed.

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98
Q

Renal-cell carcinoma, chromophobe type: Prognosis.

A

Significantly better than that of clear-cell RCC.

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99
Q

Multilocular cystic renal-cell carcinoma: Gross pathology.

A

Concysts entirely of sists filled with serous or hemorrhagic fluid.

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100
Q

Multilocular cystic renal-cell carcinoma: Histopathology.

A

Cysts are lined by one layer of epithelium.

Fibrous septa contain small clusters of clear cells.

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101
Q

Multilocular cystic renal-cell carcinoma vs. renal-cell carcinoma with cystic change.

A

The former contains no solid, expansile tumor nodules of any size.

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102
Q

Multilocular cystic renal-cell carcinoma: Prognosis.

A

Excellent.

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103
Q

Carcinoma of the collecting ducts: Location.

A

Medulla.

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104
Q

Carcinoma of the collecting ducts: Histopathologic architecture (2).

A

Tubular or tubulopapillary structures with tapered ends.

Inflamed desmoplastic stroma.

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105
Q

Carcinoma of the collecting ducts: Cytology (4).

A

High-grade nuclei.

Hobnail appearance of cells that line glands.

Many mitotic figures.

Cytoplasm may contain mucin.

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106
Q

Carcinoma of the collecting ducts: Adjacent renal tissue.

A

Shows epithelial dysplasia.

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107
Q

Carcinoma of the collecting ducts: Immunohistochemistry (5,1).

A

Positive: HMWCK, CK7, CEA, peanut agglutinin, Ulex europaeus agglutinin.

Negative: CD10.

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108
Q

Carcinoma of the collecting ducts vs. papillary renal-cell carcinoma: Immunohistochemistry.

A

Papillary renal-cell carcinoma does not express HWMCK or Ulex europaeus agglutinin.

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109
Q

Carcinoma of the collecting ducts vs. urothelial carcinoma with glandular differentiation.

A

Urothelial carcinoma with glandular differentiation is more likely if the tumor arises in the calyces or the renal pelvis.

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110
Q

Carcinoma of the collecting ducts: Prognosis.

A

Poor; one third of patients present with metastases.

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111
Q

Renal medullary carcinoma: Epidemiology.

A

Near all patients have sickle-cell trait or the disease.

Patients are typically under 40 years old.

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112
Q

Renal medullary carcinoma: Gross pathology.

A

Arises in the medulla but tends to extend into the calyces and often into the perinephric fat.

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113
Q

Renal medullary carcinoma: Histopathologic patterns (3).

A

Areas that resemble yolk-sac tumor at low power.

Areas that resemble adenoid-cystic carcinoma.

Solid sheets.

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114
Q

Renal medullary carcinoma: Cytology.

A

Poorly differentiated cells with vesicular nuclei and a large nucleolus.

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115
Q

Renal medullary carcinoma: Stroma.

A

Desmoplastic and inflamed and may contain mucin or edema.

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116
Q

Renal medullary carcinoma: Blood vessels.

A

Contain sickle cells.

Usually invaded by the tumor.

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117
Q

Renal medullary carcinoma: Immunohistochemistry (1,2).

A

Positive: Cytokeratins.

Negative: HMWCK, INI-1.

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118
Q

Renal medullary carcinoma: Prognosis.

A

Most patients are dead within 1 year.

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119
Q

Mucinous tubular and spindle-cell carcinoma:

A. Presentation.
B. Behavior.

A

A. Usually asymptomatic.

B. Low-grade carcinoma with a good prognosis.

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120
Q

Mucinous tubular and spindle-cell carcinoma: Histopathologic components (3).

A

Elongated and compressed tubules.

Spindle-shaped epithelial cells.

Extracellular mucinous Krappe.

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121
Q

Mucinous tubular and spindle-cell carcinoma: Cytology.

A

Bland cells with inconspicuous nucleoli.

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122
Q

Mucinous tubular and spindle-cell carcinoma: Immunohistochemistry (2,2).

A

Positive: Cytokeratins, AMACR.

Negative: CD10, villin (markers of proximal tubules).

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123
Q

Renal-cell carcinoma with translocations involving TFE3: Epidemiology.

A

Affects mainly children and young adults.

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124
Q

Renal-cell carcinoma with translocations involving TFE3: Location of TFE3 gene.

A

Xp11.2.

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125
Q

Renal-cell carcinoma with translocations involving TFE3: Most common translocation partners of TFE3 (2).

A

ASPL (17q25): Advanced stage at presentation.

PRCC (1q21).

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126
Q

Renal-cell carcinoma with translocations involving TFE3: Histopathologic patterns (2).

A

Papillary and nested.

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127
Q

Renal-cell carcinoma with translocations involving TFE3: Cytology (4).

A

Clear or pink cytoplasm.

Discrete cell borders.

Vesicular chromatin.

Large nucleoli.

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128
Q

Renal-cell carcinoma with translocations involving TFE3: Extracellular features (2).

A

Psammoma bodies.

Hyaline nodules.

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129
Q

Renal-cell carcinoma with translocations involving TFE3: Effect of translocation partner on histopathology (2).

A

ASPL: Cells have more cytoplasm; more psammoma bodies, more hyaline nodules.

PRCC: Less of all three.

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130
Q

Renal-cell carcinoma with translocations involving TFE3: Immunohistochemistry (4).

A

Positive: TFE3, cathepsin K, CD10, RCC.

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131
Q

Renal-cell carcinoma with t(6;11):

A. Epidemiology.
B. Prognosis.

A

A. Affects mainly children and young adults.

B. Similar to that of conventional RCC.

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132
Q

Renal-cell carcinoma with t(6;11): Gene.

A

TFEB on chromosome 6p21.

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133
Q

Renal-cell carcinoma with t(6;11): Cellular components (2).

A

Polygonal epithelioid cells with discrete borders.

Smaller cells with dark nuclei, forming clusters around hyaline matter.

Mitotic figures are rare.

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134
Q

Renal-cell carcinoma with t(6;11): Architecture.

A

Tubular, micropapillary.

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135
Q

Renal-cell carcinoma with t(6;11): Immunohistochemistry (2,2).

A

Positive: TFEB, melanoma markers.

Negative: TFE, epithelial markers.

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136
Q

Renal-cell carcinoma associated with neuroblastoma: Timing.

A

Diagnosed around 10 years after the original diagnosis of neuroblastoma.

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137
Q

Sarcomatoid differentiation in renal-cell carcinoma:

A. Setting.
B. Significance.

A

A. Can occur in any type of renal-cell carcinoma.

B. Imparts poor prognosis.

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138
Q

Sarcomatoid differentiation in renal-cell carcinoma: Histopathology (3).

A

Nondescript spindle cells.

May resemble MFH.

May resemble leiomyosarcoma or other differentiated sarcoma.

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139
Q

Sarcomatoid differentiation in renal-cell carcinoma: Immunohistochemistry.

A

May express epithelial markers and PAX8.

May not express other markers that are expressed in the better-differentiated parts of the tumor.

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140
Q

Renal tumors that contain spindle cells (4).

A

Any carcinoma with sarcomatoid differentiation.

Any carcinoma (esp. conventional) containing a scar.

Mucinous tubular and spindle-cell carcinoma.

Primary renal sarcoma.

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141
Q

Renal-cell carcinoma, unclassified: Examples of histopathology (3).

A

A tumor combining features of recognized renal tumors.

A tumor showing sarcomatoid differentiation only.

A high-grade tumor.

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142
Q

Cystic nephroma: Typical patient.

A

Middle-aged female.

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143
Q

Cystic nephroma: Presentation and behavior.

A

Asymptomatic and benign.

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144
Q

Cystic nephroma: Gross pathology.

A

Consists of many cystic spaces that do not communicate with the renal pelvis.

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145
Q

Cystic nephroma: Histopathology (2).

A

Cysts lined by bland cells.

Ovarian-type or hyalinized stroma.

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146
Q

Cystic nephroma: Disqualifying histopathologic feature.

A

Nephronic elements in the stroma.

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147
Q

Cystic nephroma: Immunohistochemistry.

A

Ovarian-type stroma expresses ER and PR.

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148
Q

Cystic nephroma: Significance of pediatric type.

A

Considered a variant of nephroblastoma.

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149
Q

Cystic nephroma vs. nephrogenic adenoma vs. metanephric adenoma.

A

Cystic nephroma: Bland cysts and ovarian-type stroma.

Nephrogenic adenoma: Renal-tubular-like proliferation outside the kidneys.

Metanephric adenoma: Small acini consisting of small, dark cells.

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150
Q

Mixed epithelial and stromal tumor: Epidemiology and putative etiology.

A

Mostly in females; estrogen imbalance.

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151
Q

Mixed epithelial and stromal tumor: Gross pathology (2).

A

Central location in the kidney.

Contains cystic (epithelial) and solid (stromal) areas.

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152
Q

Mixed epithelial and stromal tumor vs. cystic nephroma.

A

MEST
− Epithelial component: Tubules as well as cells.
− Stromal component: Grossly evident; histologically more variable (e.g. fat, smooth muscle).

Both tumors are considered part of the same spectrum.

153
Q

Tubulocystic carcinoma: Typical patient.

A

Middle-aged man.

154
Q

Tubulocystic carcinoma: Gross pathology (3).

A

Entirely cystic, resembling bubble wrap.

Well circumscribed but has no capsule.

155
Q

Tubulocystic carcinoma: Histopathology.

A

Closely packed cysts separated by delicate septa or a fibrotic stroma.

No tumor cells in those septa.

156
Q

Tubulocystic carcinoma: Cytology.

A

Cells resemble hobnails and contain large nucleoli.

157
Q

Tubulocystic carcinoma: Immunohistochemistry (2,1).

A

Positive: CD10, AMACR.

Negative: CD7.

158
Q

Tubulocystic carcinoma vs. multilocular cystic renal-cell carcinoma.

A

Multilocular cystic renal-cell carcinoma: Nests of clear cells in the septa.

159
Q

Clear-cell tubulopapillary renal-cell carcinoma: Behavior.

A

Low grade, low stage.

160
Q

Clear-cell tubulopapillary renal-cell carcinoma: Gross pathology.

A

Cystic; fibrous capsule.

161
Q

Clear-cell tubulopapillary renal-cell carcinoma: Architecture (3).

A

Papillae, tubules, cysts.

162
Q

Clear-cell tubulopapillary renal-cell carcinoma: Cytology.

A

Clear cytoplasm.

Bland nuclei (Fuhrman grade 1 or 2).

Nuclei are oriented toward the lumens and away from the basement membranes.

163
Q

Clear-cell tubulopapillary renal-cell carcinoma: Cytogenetics.

A

No +7, +17, -Y.

No -3p.

164
Q

Clear-cell tubulopapillary renal-cell carcinoma: Immunohistochemistry (2,2).

A

Positive: CK7 (membranous), CA9.

Negative: AMACR, CD10.

165
Q

Hereditary leiomyomatosis:

A. Inheritance.
B. Tumors (3).

A

A. Autosomal dominant.

B. Leiomyomas, uterine leiomyosarcoma, renal-cell carcinoma.

166
Q

Hereditary leiomyomatosis: Gene and its location.

A

Fumarate hydratase on 1q42.

167
Q

Renal-cell carcinoma of hereditary leiomyomatosis: Architecture.

A

Similar to that of papillary RCC, type II.

168
Q

Renal-cell carcinoma of hereditary leiomyomatosis: Cytology.

A

Large nucleus contains a very large, red nucleolus with a clear halo.

169
Q

Renal-cell carcinoma of hereditary leiomyomatosis: Immunohistochemistry.

A

Positive: 2SC (2-succinylcysteine).

170
Q

Renal-cell carcinoma of hereditary leiomyomatosis: Prognosis.

A

Poor.

171
Q

Birt-Hogg-Dubé syndrome: Skin tumors (3).

A

Fibrofolliculoma.

Trichodiscoma.

Acrochordons.

172
Q

Birt-Hogg-Dubé syndrome: Gene, location, product.

A

BHD, 17p11.2, folliculin.

173
Q

Renal-cell carcinoma associated with Birt-Hogg-Dubé syndrome: Histopathology (3).

A

Oncocytoma + chromophobe RCC.

Oncocytosis outside the tumor.

Clear cells without wrinkled nuclei.

174
Q

Renal-cell carcinoma associated with Birt-Hogg-Dubé syndrome: Immunohistochemistry (3).

A

Positive: CK7, CD117, parvalbumin.

175
Q

Renal-cell carcinoma associated with Birt-Hogg-Dubé syndrome: Prognosis.

A

Excellent; no aggressive behavior reported.

176
Q

SDH syndrome: Tumors (3).

A

Paraganglioma / pheochromocytoma.

Gastrointestinal stromal tumor.

Renal-cell carcinoma.

177
Q

SDH syndrome: Genes.

A

SDHB, SDHC, SDHD: Subunits of succinate dehydrogenase in Krebs’ cycle.

178
Q

Renal-cell carcinoma of SDH syndrome: Mean age at presentation.

A

33 years.

179
Q

Renal-cell carcinoma of SDH syndrome: Histopathology (2).

A

Mutation in SDHB: Consists of oncocytes containing large vacuoles and bland nuclei.

Mutation in SDHC or SDHD: Resembles clear-cell RCC.

180
Q

Renal-cell carcinoma of SDH syndrome: Prognosis.

A

May be aggressive, especially in younger patients.

181
Q

Renal-cell carcinoma associated with acquired cystic disease: Risk factors (2).

A

Male gender.

Long-term dialysis.

182
Q

Renal-cell carcinoma associated with acquired cystic disease: Architecture.

A

Mixture of sheets, nests, tubulopapillary areas, and cystic areas.

183
Q

Renal-cell carcinoma associated with acquired cystic disease: Cytology (2).

A

Eosinophilic cytoplasm.

Nuclei of Fuhrman grade 3.

184
Q

Renal-cell carcinoma associated with acquired cystic disease: Intracellular features (2).

A

Microlumens, hemosiderin.

185
Q

Renal-cell carcinoma associated with acquired cystic disease: Extracellular feature.

A

Oxalate crystals.

186
Q

Renal-cell carcinoma associated with acquired cystic disease: Immunohistochemistry (2,3).

A

Positive: AMACR, CD10.

Negative: CA9, PAX8, CK7.

187
Q

Renal medullary interstitial-cell tumor / medullary fibroma: Presentation.

A

Asymptomatic; found incidentally.

188
Q

Renal medullary interstitial-cell tumor / medullary fibroma: Histopathology.

A

Medullary tubules entrapped in a paucicellular matrix that is collagenous or myxoid.

189
Q

Renal medullary interstitial-cell tumor / medullary fibroma: Cell of origin.

A

Renal medullary interstitial cell, which helps to control the blood pressure.

190
Q

Juxtaglomerular-cell tumor: Presentation.

A

Hypertension.

191
Q

Juxtaglomerular-cell tumor: Laboratory findings (3).

A

Elevated plasma renin.

Elevated aldosterone.

Hypokalemia.

192
Q

Juxtaglomerular-cell tumor: Location.

A

Renal cortex.

193
Q

Juxtaglomerular-cell tumor: Architecture (3).

A

Trabeculae or glomeruloid formations.

Myxoid stroma containing lymphocytes.

Hemangiopericytoma-like vasculature.

194
Q

Juxtaglomerular-cell tumor: Cytology (3).

A

Polygonal or spindle shape.

Moderate or abundant pink, granular cytoplasm.

Bland nuclei.

195
Q

Juxtaglomerular-cell tumor: Immunohistochemistry (2,3).

A

Positive: CD31, actins.

Negative: Cytokeratins, desmin, melanoma markers.

196
Q

Juxtaglomerular-cell tumor: Electron microscopy.

A

Rhomboid crystals of renin.

197
Q

Juxtaglomerular-cell tumor: Prognosis.

A

Never reported to recur or metastasize.

198
Q

Nephroblastoma: Age group.

A

Children below the age of 6.

199
Q

Nephroblastoma: Syndromes (4).

A

WAGR.

Denys-Drash.

Beckwith-Wiedemann.

Familial neuroblastoma.

200
Q

WAGR syndrome:

A. Components.
B. Mutation.

A

A. Wilms’ tumor, aniridia, genital anomalies, mental retardation.

B. del(11p13), including WT1.

201
Q

Denys-Drash syndrome: Mutation.

A

Point mutation in WT1.

202
Q

Beckwith-Wiedemann: Gene.

A

WT2 on 11p15.

203
Q

Nephroblastoma: Typical presentation.

A

Abdominal mass or tenderness.

204
Q

Nephroblastoma:

A. Histological components (3).
B. Variation.

A

A. Blastemal, stromal, epithelial.

B. Only two or only one component may be represented.

205
Q

Nephroblastoma: Blastema.

A

Consists of small, round, blue cells with coarse chromatin.

206
Q

Nephroblastoma: Stroma (2).

A

Myxoid or fibromyxoid.

May show differentiation toward skeletal muscle, cartilage, etc.

207
Q

Nephroblastoma: Epithelial component (2).

A

Poorly or well-formed tubules and papillae.

Squamous or mucinous foci may be seen.

208
Q

Nephroblastoma: Definition of nuclear anaplasia (3).

A

Polyploidy

− or −

Multipolar mitotic figures

− or −

Greater-than-threefold variation in nuclear size.

209
Q

Nephroblastoma: Significance of nuclear anaplasia.

A

Predicts responsiveness to chemotherapy.

210
Q

Nephroblastoma: Immunohistochemistry for WT1.

A

Blastema and undifferentiated epithelium: Diffuse expression.

Stroma: Little or no expression.

Differentiated epithelium: Variable.

211
Q

Nephroblastoma: Distinction from other tumors of small, round, blue cells (2).

A

IHC: Expression of WT-1 by nephroblastoma.

EM: Variety of organelles in blastemal cells of nephroblastoma.

212
Q

Nephroblastoma: Sites of metastasis (3).

A

Lymph nodes, liver, lung.

213
Q

Nephroblastoma: Prognosis (2).

A

About 90% of cases get cured.

Younger patients do better.

214
Q

Nephrogenic rest:

A. Significance.
B. State of multiplicity.

A

A. Increased likelihood of contralateral nephroblastoma.

B. Nephroblastomatosis.

215
Q

Nephroblastoma: Histopathology (2).

A

Perilobar type: Mainly blastema and tubules; very little stroma.

Intralobar type: Rich in stroma.

216
Q

Cystic partially differentiated nephroblastoma: Histopathology (3).

A

Epithelial-lined cysts or unlined cyst-like structures.

Septa may contain nephroblastomatous epithelium.

No expansile solid component.

217
Q

Cystic partially differentiated nephroblastoma: Variant.

A

Pediatric cystic nephroma contains no nephroblastomatous elements.

218
Q

Cystic partially differentiated nephroblastoma vs. Cystic nephroblastoma.

A

The latter has an expansile solid component.

219
Q

Mesoblastic nephroma: Epidemiology (2).

A

Patients are usually no more than 3 months old.

Most frequent congenital renal neoplasm.

220
Q

Mesoblastic nephroma: Presentation.

A

Abdominal mass.

221
Q

Mesoblastic nephroma: Behavior (2).

A

Excellent if completely resected.

222
Q

Mesoblastic nephroma: Location.

A

Renal sinus.

223
Q

Mesoblastic nephroma: Gross pathology (2).

A

Classic: Small; firm; resembles leiomyoma.

Cellular: Large; soft; cystic; focally hemorrhagic and necrotic.

224
Q

Mesoblastic nephroma: Histopathology of classic type.

A

Resembles benign fibromatosis.

225
Q

Mesoblastic nephroma: Histopathology of cellular type.

A

Sheets or vague fascicles of densely packed, plump, mitotically active cells.

Same as infantile fibrosarcoma.

226
Q

Mesoblastic nephroma: Immunohistochemistry (2).

A

Positive: SMA, vimentin.

227
Q

Mesoblastic nephroma: Electron microscopy.

A

Much endoplasmic reticulum.

228
Q

Mesoblastic nephroma: Translocation.

A

t(12;15)(p13;q25).

229
Q

Mesoblastic nephroma vs. cystic nephroma.

A

Mesoblastic nephroma: Spindle-cell mesenchymal tumor in infants.

Cystic nephroma: Bland cysts and ovarian-type stroma; occurs mainly in women.

230
Q

Mesoblastic nephroma vs. metanephric adenoma.

A

Mesoblastic nephroma: Spindle-cell mesenchymal tumor in infants.

Metanephric adenoma: Small acini consisting of small, dark cells; occurs in adults.

231
Q

Clear-cell sarcoma: Age group.

A

Between 6 months and 5 years.

232
Q

Clear-cell sarcoma: Cytology of classic type (3).

A

Cytoplasm: Pale.

Nucleus: Vesicular.

Nucleolus: Inconspicuous.

233
Q

Clear-cell sarcoma: Histopathology of classic type (2).

A

Cells form cords that are separated by regularly spaced fibrovascular arcades.

Normal renal structures are entrapped at the periphery of the tumor.

234
Q

Clear-cell sarcoma: Variants (3).

A

Myxoid, sclerosing, epithelioid, many others.

235
Q

Clear-cell sarcoma: Immunohistochemistry (2,3).

A

Positive: Vimentin, nerve-growth-factor receptor.

Negative: Cytokeratins, neural markers, WT1.

236
Q

Clear-cell sarcoma: Prognosis.

A

Very aggressive.

237
Q

Rhabdoid tumor: Age at presentation.

A

Usually less than 2 years.

238
Q

Rhabdoid tumor:

A. Laboratory findings.
B. Associated tumor.

A

A. Hypercalcemia, hematuria.

B. PNET of the posterior fossa.

239
Q

Rhabdoid tumor: Cytology.

A

Cytoplasm: Abundant, pink; often contains large eosinophilic globular inclusions.

Nucleus: Vesicular; thick membrane.

Nucleolus: Large.

240
Q

Rhabdoid tumor: Immunohistochemistry (1,1).

A

Positive: Epithelial markers.

Negative: INI-1.

241
Q

Rhabdoid tumor: Electron microscopy.

A

Intermediate filaments make up the cytoplasmic inclusions.

242
Q

Rhabdoid tumor: Mutation.

A

Inactivation of INI-1 (hSNF5) on chromosome 22.

243
Q

Rhabdoid tumor: Prognosis.

A

Bad enough in older children; dismal in infants.

244
Q

Metastasis to the kidney: Leading primary sites (5).

A

Lung.

Melanoma.

Gastrointestinal tract.

Gonads.

Other kidney.

245
Q

Metastasis to the kidney: Location.

A

Cortex or medulla (or both).

246
Q

Diseases of podocytes: Laboratory finding.

A

Proteinuria.

247
Q

Minimal-change disease: Epidemiology (2).

A

Most patients are young children.

Adults can be affected, particularly secondary to nephrotoxins.

248
Q

Minimal-change disease: Histopathology.

A

Normal by H&E and by special stains.

249
Q

Minimal-change disease: Electron microscopy (4).

A

Loss of foot processes.

Extensive microvillous transformation.

Generally normal glomerular basement membrane.

No deposits.

250
Q

Focal-segmental glomerulosclerosis: Laboratory findings (2).

A

Nephrotic syndrome.

Azotemia.

251
Q

Focal-segmental glomerulosclerosis: Etiologies (5).

A

Idiopathic.

Genetic.

Immunologic.

Drugs.

HIV.

252
Q

Focal-segmental glomerulosclerosis: Electron microscopy (4).

A

Loss of foot processes.

Microvillous transformation.

Wrinkled glomerular basement membranes.

HIV-associated FSGS: Tubuloreticular inclusions may be seen.

253
Q

Renal diseases of collagen: Affected parts of the nephron.

A

Glomerular basement membrane and/or mesangium.

254
Q

Disorders of collagen, type IV (3).

A

Benign familial hematuria: Hematuria only; no progression.

Thin-basement-membrane disease: Hematuria; usually no progression.

Alport’s syndrome: Hematuria and proteinuria.

255
Q

Alport’s syndrome: Additional manifestations (2).

A

X-linked type: Ocular abnormalities, sensorineural deafness.

256
Q

Alport’s syndrome: Electron-microscopic changes in the glomerular basement membrane (3).

A

Variations in thickness, sometimes with breaks.

“Basket-weave” alternation of dense and lucent areas.

Multilamellation and scalloping.

257
Q

Alport’s syndrome: Prognosis.

A

X-linked Alport’s syndrome: End-stage renal disease in 90% of patients by age 40.

258
Q

Alport’s syndrome: Mutations (2).

A

Most cases of X-linked disease: COL4A5.

Some cases of AR or AD disease: COL4A3 or COL4A4.

259
Q

Alport’s syndrome: Histopathology of early lesion.

A

Normal.

260
Q

Alport’s syndrome: Histopathology of the glomerulus in a late lesion.

A

Irregular thickness of glomerular basement membrane.

Segmental solidification of the glomerular tuft (as in FSGS).

261
Q

Alport’s syndrome: Histopathology of the renal interstitium in a late lesion.

A

Fibrosis with tubular atrophy.

Foam cells.

Sclerosis and hyalinosis of arterioles.

262
Q

Glomerular basement membrane: Normal thickness.

A

At least 200 nm.

263
Q

Thrombotic microangiopathies: Components (2).

A

Endothelial injury.

Thrombosis.

264
Q

Thrombotic microangiopathies: Examples (6).

A

Hemolytic-uremic syndrome.

TTP.

Malignant hypertension.

Renal crisis of scleroderma.

Antiphospholipid syndrome.

Iatrogenic thrombotic microangiopathy.

265
Q

Thrombotic microangiopathies: Histopathology of acute glomerular disease (2).

A

Intracapillary thrombi.

Thickened walls of capillaries.

266
Q

Thrombotic microangiopathies: Histopathology of chronic glomerular disease (2).

A

Membranoproliferative features with double contours (“tram-track” appearance).

Mesangial and global sclerosis.

267
Q

Thrombotic microangiopathies: Histopathology of chronic interstitial disease (2).

A

Fibrosis.

Tubular atrophy.

268
Q

Thrombotic microangiopathies: Histopathology of malignant hypertension (2).

A

“Onion-skin” appearance of blood vessels.

Extreme duplication of elastic lamina.

269
Q

ANCA-mediated vasculitis of small vessels: Presentation (3).

A

Hematuria, proteinuria, rapid progression to renal failure.

Signs of systemic vasculitis.

Dermatological disease.

270
Q

ANCA-mediated vasculitis of small vessels: Types of ANCA.

A

p-ANCA: Myeloperoxidase.

c-ANCA: Proteinase 3.

271
Q

ANCA-mediated vasculitis of small vessels: Pathogenesis.

A

The ANCAs activate neutrophils, which injure the endothelium.

272
Q

ANCA associated with

A. Wegener’s granulomatosis.
B. Microscopic polyangiitis.
C. Churg-Strauss syndrome.
D. Renal-limited glomerulonephritis.

A

A. c-ANCA.

B,C,D. p-ANCA.

273
Q

ANCA-mediated vasculitis of small vessels: Glomerular changes (2).

A

Cellular crescents progress to segmental glomerular scars.

Bowman’s capsule may be occluded or disrupted.

274
Q

ANCA-mediated vasculitis of small vessels:

A. Contents of cellular crescents (4).
B. Contents of glomerular scars.

A

A. Parietal epithelial cells, podocytes, inflammatory cells, fibrinoid necrotic débris.

B. More fibroblasts and fibrin.

275
Q

ANCA-mediated vasculitis of small vessels: Extraglomerular vascular changes (2).

A

Leukocytoclastic vasculitis.

Transmural arteritis with fibrinoid necrosis.

276
Q

ANCA-mediated vasculitis of small vessels: Other changes (3).

A

Interstitial inflammation.

Interstitial granulomas in Wegener’s granulomatosis.

Acute tubular injury in aggressive forms.

277
Q

Anti-glomerular-basement-membrane disease: Epidemiology (2).

A

Adolescent males.

Middle-aged females.

278
Q

Anti-glomerular-basement-membrane disease: Presentation (2).

A

Acute renal failure with hematuria and proteinuria.

Pulmonary hemorrhage.

279
Q

Anti-glomerular-basement-membrane disease: Pathogenesis.

A

Antibodies against the noncollagenous-1 domain of the α₃ chain of collagen, type IV.

280
Q

Anti-glomerular-basement-membrane disease: Relevance to HLA type.

A

Strongly associated with HLA-DR and HLA-DQ.

281
Q

Anti-glomerular-basement-membrane disease: Histopathology (4).

A

Cellular crescents involving all glomeruli.

Progression of crescents to segmental glomerular scars.

Interstitial inflammation.

Acute tubular injury in aggressive forms.

282
Q

Anti-glomerular-basement-membrane disease: Serologic diagnosis (3).

A

DIF: Linear pattern of IgG on the GBM.

IIF: Anti-GBM antibodies in patient’s serum react with normal kidney.

No ANCAs.

283
Q

Immune-complex-mediated glomerular diseases: Types (3).

A

Membranous glomerulopathy.

Infection-mediated glomerular diseases.

IgA-mediated glomerular diseases.

284
Q

Membranous glomerulopathy: Presentation.

A

Nephrotic syndrome progressing to ESRD in 30% of cases.

285
Q

Membranous glomerulopathy: Etiologies (5).

A

Primary: Idiopathic, congenital.

Secondary: HCV, HBV, syphilis.

Autoimmune diseases.

GVHD.

Neoplasia.

286
Q

Membranous glomerulopathy: Pathogenesis of idiopathic form.

A

Antibodies to phospholipase-A2 receptors in podocytes.

287
Q

Membranous glomerulopathy: Histopathology (3).

A

Thickening of the GBM.

Mesangial expansion and proliferation.

Glomerulitis in cases associated with malignancy.

288
Q

Membranous glomerulopathy: Special stain.

A

Silver stain: Spikes and/or gaps in the GBM.

289
Q

Membranous glomerulopathy: Direct immunofluorescence (2).

A

GBM: IgG, C3, kappa, lambda in granular pattern.

Tubular basement membrane: Deposits seen in secondary forms.

290
Q

Membranous glomerulopathy: Type of IgG (2).

A

Primary: Mainly IgG4.

Neoplastic: IgG1, IgG2.

291
Q

Membranous glomerulopathy: Electron microscopy

A

Extensive effacement of foot processes.

Subepithelial electron-dense deposits.

Secondary forms: Deposits in mesangium, tubular basement membrane.

292
Q

Infection-mediated glomerular disease: Laboratory findings (3).

A

Hematuria.

Proteinuria.

Hypocomplementemia.

293
Q

Infection-mediated glomerular disease: Presentation.

A

Nephritic syndrome.

294
Q

Infection-mediated glomerular disease: Etiologies (6).

A

Streptococci.

MRSA.

Viruses, rickettsiae, fungi, parasites.

295
Q

Infection-mediated glomerular disease: Special stain.

A

Trichrome stain may show subepithelial humps.

296
Q

Infection-mediated glomerular disease: Electron microscopy (2).

A

Subepithelial humps with effacement of overlying foot processes.

Subendothelial, intramembranous, and mesengial electron-dense deposits.

297
Q

Infection-mediated glomerular disease: Direct immunofluorescence (2).

A

Granular pattern in basement membrane and mesangium

− Most cases: IgG and C3.
− MRSA-related: IgA and C3.

298
Q

IgA-related nephropathies: Epidemiology.

A

Common in Asians; rare in blacks.

299
Q

IgA-related nephropathies: Clinical presentations (2).

A

Asymptomatic: Occult hematuria and proteinuria.

Symptomatic: Nephritic syndrome.

300
Q

IgA-related nephropathies:

A. Laboratory finding.
B. Prognosis.

A

A. Normal serum complement levels.

B. Recurs in 30% of transplants.

301
Q

IgA-related nephropathies: Pathophysiology (2).

A

Abnormal glycosylation of IgA1.

Increased production of IgA.

302
Q

IgA-related nephropathies: Relevance to HLA type.

A

Associated with HLA-DQ.

303
Q

IgA-related nephropathies: Histopathology (2).

A

Variable; may resemble other glomerular diseases.

Diagnosis requires immunofluorescence.

304
Q

IgA-related nephropathies: Direct immunofluorescence.

A

IgA, kappa, lambda, C3, generally in a mesangial pattern.

305
Q

IgA-related nephropathies: Electron microscopy (3).

A

Mesangium: Deposits.

Basement membrane: Variable thinning; deposits in some cases.

Foot processes: Variable effacement.

306
Q

IgA-related nephropathy vs. ANCA-associated vasculitis.

A

Significant mesangial proliferation favors an IgA-related nephropathy.

307
Q

IgA-related nephropathy vs. FSGS.

A

Demonstration of mesangial deposits (by DIF or EM) favors an IgA-related nephropathy.

308
Q

Membranoproliferative glomerulonephritis: Classification.

A

Types I and III: Mediated by immune complexes.

Type II: Mediated by complement; now known as dense-deposit disease.

309
Q

Immune-complex-mediated membranoproliferative glomerulonephritis:

A. Epidemiology.
B. Presentation.

A

A. Children and young adults.

B. Both nephrotic syndrome and nephritic syndrome.

310
Q

Immune-complex-mediated membranoproliferative glomerulonephritis:

A. Laboratory finding.
B. Etiologies (2).

A

A. Decreased serum complement.

B. HCV; idiopathic.

311
Q

Immune-complex-mediated membranoproliferative glomerulonephritis, type I: Constant histopathologic feature.

A

Double contours formed by interposition of mesangial cytoplasm and deposits.

312
Q

Immune-complex-mediated membranoproliferative glomerulonephritis, type III of Burkholder: Histopathology.

A

Features of MPGN I + mesangial proliferation and diffuse thickening of capillary walls.

313
Q

Immune-complex-mediated membranoproliferative glomerulonephritis, type III of Anders and Strife: Histopathology (2).

A

Features of MPGN I + irregular thickening of capillary walls.

Less mesangial proliferation than in type III of Burkholder.

314
Q

Immune-complex-mediated membranoproliferative glomerulonephritis, type III of Anders and Strife: Special stain.

A

Silver stain shows non-staining (“moth-eaten”) areas of basement membrane.

315
Q

Immune-complex-mediated membranoproliferative glomerulonephritis: Direct immunofluorescence.

A

“Lumpy-bumpy” deposits of IgG and C3 in mesangium and capillary walls.

316
Q

Electron microscopy of immune-complex-mediated membranoproliferative glomerulonephritis:

A. Type I.
B. Type III of Burkholder.
C. Type III of Anders and Strife.

A

A. Double contours.

B. Double contours and numerous subepithelial deposits.

C. Double contours and deposits all throughout the basement membrane.

317
Q

C3 glomerulopathies:

A. Age group.
B. Presentation.

A

A. Children and young adults.

B. Both nephrotic and nephritic syndromes.

318
Q

C3 glomerulopathies: Pathogenesis.

A

Dysfunction of the alternative pathway of complement.

319
Q

C3 glomerulopathies: Type (2).

A

C3 glomerulonephritis: Similar to MPGN I and III, but without immune complexes.

Dense-deposit disease.

320
Q

C3 glomerulopathies: Histopathology (2).

A

C3 glomerulonephritis: Variable.

DDD: Irregular thickening of basement membrane; mild mesangial proliferation.

321
Q

C3 glomerulopathies: Special stains (2).

A

Silver stain and PAS

− C3 glomerulonephritis: Double contours.
− DDD: “Moth-eaten” GBM.

322
Q

C3 glomerulopathies: Direct immunofluorescence (2).

A

C3 glomerulonephritis: Granular deposition of C3.

DDD: “Garland” pattern of C3.

323
Q

C3 glomerulopathies: Predictors of poor prognosis (3).

A

Older age at presentation.

Higher creatinine at presentation.

Extracapillary proliferation.

324
Q

Lupus nephritis: Frequency (2).

A

Occurs in 80% of patients with lupus.

Presenting manifestation in 20% of lupus patients.

325
Q

Lupus nephritis: Class I (2).

A

Normal glomeruli by histology.

Mesangial deposits by IF or EM.

326
Q

Lupus nephritis: Class II (2).

A

Mesangial expansion and proliferation by histology.

Mesangial deposits by IF or EM.

327
Q

Lupus nephritis: Class III (2).

A

Segmental or global focal endocapillary proliferation involving less than half of glomeruli.

Mesangial and subendothelial deposits by EM.

328
Q

Lupus nephritis: Class IV (2).

A

Segmental or global diffuse endocapillary proliferation involving at least half of glomeruli.

Mesangial and subendothelial deposits by EM.

329
Q

Lupus nephritis: Class V (2).

A

Thickened GBM by histology.

Subepithelial deposits by EM.

330
Q

Lupus nephritis: Class VI.

A

Global sclerosis involving more than half of glomeruli.

331
Q

Lupus nephritis: Definition of endocapillary proliferation (3).

A

Proliferation of mesangial and endothelial cells.

Inflammatory cells may be marginated.

Glomerulus appears lobulated and hypercellular.

332
Q

Lupus nephritis: “Wire loops”.

A

Large subendothelial deposits.

Best seen with trichrome stain.

333
Q

Lupus nephritis: Definition of extracapillary proliferation.

A

Cellular crescents.

334
Q

Lupus nephritis: Interstitial changes (2).

A

Acute: Plasmacytic inflammation and edema.

Chronic: Fibrosis and tubular atrophy.

335
Q

Lupus nephritis: Vascular changes.

A

Variable.

336
Q

Lupus nephritis: Silver stain (2).

A

Classes III and IV: Double contours.

Class V: Spikes and holes in the GBM.

337
Q

Lupus nephritis: Direct immunofluorescence (2).

A

Granular distribution of all immunoglobulins and complement.

C1q is always present.

338
Q

Electron microscopy of lupus nephritis:

A. Appearance of deposits.
B. Another abnormal structure.

A

A. May resemble fingerprints.

B. Tubuloreticular inclusions in endothelial cells.

339
Q

Diabetic nephropathy: Early sign.

A

Microalbuminuria: Occurs more than 5 years before onset of diabetes.

340
Q

Diabetic nephropathy: Stage 1 (2).

A

Light microscopy: Normal glomeruli.

Em: Thickened GBM.

341
Q

Diabetic nephropathy: Stage 2 (2).

A

LM: Diffuse mesangial expansion.

EM: Thickened GBM; increased collagen in mesangium.

342
Q

Diabetic nephropathy: Stage 3 (2).

A

LM: Diffuse nodular glomerulosclerosis (Kimmelstiel-Wilson lesion); abundant hyalinosis.

343
Q

Diabetic nephropathy: Stage 4.

A

More than half of glomeruli are sclerotic.

344
Q

Diabetic nephropathy: Types of hyaline lesion (3).

A

Fibrin caps (hyalinosis of capillaries).

Capsular drops.

Arteriolar hyalinosis.

345
Q

Diabetic nephropathy: Interstitial changes (2).

A

Fibrosis and tubular atrophy.

346
Q

Diabetic nephropathy: Vascular change.

A

Arteriolar hyalinosis involving afferent and efferent arterioles.

347
Q

Diabetic nephropathy: Special stain.

A

Silver stain emphasizes sclerotic nodules.

348
Q

Diabetic nephropathy: Direct immunofluorescence (2).

A

GBM, tubular BM: Linear IgG and albumin.

Areas of glomerular and vascular hyalinosis: C3 and IgM.

349
Q

Electron microscopy of diabetic nephropathy:

A. Glomerular basement membrane.
B. Mesangium.

A

A. Diffuse and sometimes massive thickening.

B. Sclerosis; collagen fibrils.

350
Q

Diabetic nephropathy vs. anti-GBM disease (2).

A

Diabetic nephropathy:

− Usually has no crescents.
− Linear deposition of albumin.

351
Q

Fabry’s disease: Affected organs (4).

A

Skin.

Nervous system.

Kidneys.

Gastrointestinal tract.

352
Q

Fabry’s disease:

A. Inheritance.
B. Gene and its product.
C. Pathogenesis.

A

A. X-linked.

B. AGAL; α-galactosidase A.

C. Accumulation of lipids in cells.

353
Q

Fabry’s disease:

A. Histopathology.
B. Special stain.

A

A. Lipid vacuoles in podocytes, tubular epithelial cells, and endothelial cells.

B. Toluidine blue reveals the inclusions.

354
Q

Fabry’s disease: Electron microscopy.

A

Lipids have lamellated appearance (zebra bodies).

355
Q

Fabry’s disease: Drug that can cause similar inclusions.

A

Chloroquine.

356
Q

Renal amyloidosis: Presentation.

A

Nephrotic syndrome.

357
Q

Renal amyloidosis: Immunoglobulin-derived amyloid (2).

A

AL, especially λ light chain.

Heavy chains.

358
Q

Renal amyloidosis:

A. Most common genetic form.
B. Associated with chronic inflammation.
C. Associated with dialysis.

A

A. α-Fibrinogen.

B. AA (serum amyloid A).

C. β₂-microglobulin.

359
Q

Renal amyloidosis: Other forms of amyloid (2).

A

Leukocyte chemotactic factor 2.

Transthyretin.

360
Q

Renal amyloidosis: Histopathology (4).

A

Glomeruli: Thickening of basement membranes; mesangial nodules.

Tubules: Thickening of basement membranes.

Arterioles: Intimal nodules.

361
Q

Renal amyloidosis: Electron microscopy.

A

Non-branching, randomly organized fibrils, 9-11 nm in diameter.

362
Q

Renal amyloidosis vs. light-chain-deposition disease.

A

In the latter, there are finely granular, electron-dense deposits.

363
Q

Acute tubular injury:

A. Presentation.
B. Laboratory finding.

A

A. Acute renal failure with oliguria or anuria.

B. Granular casts.

364
Q

Acute tubular injury: Causes.

A

Ischemia (90%).

Nephrotoxins.

365
Q

Acute tubular injury: Most vulnerable part of nephron.

A

The distal part.

366
Q

Acute tubular injury: Histopathology (4).

A

Tubular epithelial cells:
− Loss of brush border.
− Necrosis in a minority of tubules.
− Regenerative changes.

Glomeruli and blood vessels: No change.

367
Q

Acute tubular injury: Special stain.

A

PAS reveals loss of brush borders.

368
Q

Drug-induced interstitial nephritis: Classic triad.

A

Hypersensitivity reaction.

Maculopapular rash.

Eosinophilia.

369
Q

Drug-induced interstitial nephritis: Laboratory findings (3).

A

Mild proteinuria.

Microscopic hematuria.

Eosinophiluria.

370
Q

Drug-induced interstitial nephritis: Causes (4).

A

NSAIDs.

Antimicrobials.

Diuretics.

Others.

371
Q

Renal dysplasia: Histopathology (3).

A

Disorganized renal parenchyma containing cartilage.

Dysplastic ducts lined by columnar epithelium and surrounded by spindle cells.

Cystic spaces lined by flat epithelium.

372
Q

Drug-induced interstitial nephritis: Electron microscopy.

A

NSAIDs: Diffuse injury to podocytes, with a effacement of foot processes.

373
Q

Acute obstructive nephropathy: Causes (3).

A

Cast nephropathies:
− Myeloma.
− Myoglobin.
− Hemoglobin.

374
Q

Myeloma cast nephropathy: Histopathology (3).

A

Fractured or crystalline-appearing casts surrounded by giant cells.

Reactive epithelial cells in the affected tubules.

Interstitial fibrosis and lymphocytic inflammation.

375
Q

Myeloma cast nephropathy: Special stain.

A

Negative for PAS (Tamm-Horsfall casts are positive).

376
Q

Hypertensive nephropathy: Other histologic changes (3).

A

Glomeruli: Global sclerosis.

Tubules: Atrophy.

Interstitium: Fibrosis.

377
Q

Hypertensive nephropathy: Histologic changes in arteries (3).

A

Intimal fibrosis, medial hypertrophy, duplication of the elastic lamina.

378
Q

Hypertensive nephropathy: Histologic change in arterioles.

A

Hyalinization.

379
Q

Drug-induced interstitial nephritis: Histopathology (3).

A

Interstitial edema and inflammation, often including eosinophils.

Inflammation of tubules in the active phase.

Granulomas can be seen.

AP reviewer (17 decks)

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