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Surgical Kidney Flashcards

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1
Q

Name 5 familial cancer syndromes associated with renal neoplasms

A
  • Von Hippel-Lindau (VHL) - Hereditary papillary renal cell carcinoma syndrome (HRPC) - Birt-Hogg-Dube syndrome - Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) - Tuberous sclerosis
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2
Q

Name 5 familial pediatric renal tumor syndromes

A
  • WAGR (Wilms, aniridia, genitourinary malformations, mental retardation) - Denys-Drash - Beckwith-Wiedemann syndrome - Familial nephroblastoma - Trisomy 18, Perlman Syndrome, Bloom syndrome, Frasier syndrome
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3
Q

Von Hippel Lindau: Gene involved, gene product, pathway, type of renal tumor, other associations

A

Gene: VHL (3p25) Product: pVHL pathway: Hypoxia-inducible factor (HIF) tumor: Clear cell RCC others: cysts, CNS/retinal hemangioblastoma, pheochromocytoma, pancreatic cysts, pancreatic endrocrine tumor

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4
Q

Hereditary papillary renal cell carcinoma: Gene, gene product, pathway, type of tumor, others

A

Gene: c-MET (7q31) Product: MET tyrosine kinase Pathway: MET/hepatocyte growth factor Tumor: Papillary renal cell carcinoma, type 1 others: no

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5
Q

Birt-Hogg-Dube: gene, gene product, pathway, type of tumor, others

A

Gene: BHD (17), product: folliculin pathway: MTOR (mammalian target of rapamycin) tumor: Oncocytic tumors (chromophobe, oncocytoma, hybrid), other RCC Others: cutaneous lesions, lung cysts, pneumothorax, lipomas

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6
Q

Hereditary leiomyomatosis and RCC: gene/gene product, pathway, tumors, others

A

gene: Fumarate hydratase (1q)/fumarate hydratase gene product pathways involed: HIF tumor: High-grade RCC with papillary, tubular, solid architecture others: leiomyomas of skin/uterus

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7
Q

Tuberous sclerosis: gene/product, pathway, tumors, others

A

gene: Tuberous sclerosis complex (TSC1, chromosome 9, TSC2, chromosome 16) products: hamartin, tuberin pathway: MTOR tumors: AML, RCC others: lymphangioleioyomatosis of lung, clear cell tumors of lung, pancreas, uterus, pecomas, subependymomas, giant cell astrocytoma

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8
Q

Renal malakoplakia: what are gross features

A
  • Pelvicalyceal system outlined by thick bands of friable/necrotic tissue - Irregular yellow masses centered on renal medulla - Hydronephrosis or pyelonephrosis - Renal calculi (especially staghorn)
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9
Q

Renal malakoplakia/xanthogranulomatous pyelonephritis: What are microscopic features

A
  • Aggregates of histocytes with eosinophilic cytoplasm (von Hansemann histocytes) - Cytoplasm is PAS+ - Some contain concentrically lamellar, basophilic, calcified inclusions (Michaelis-Gutmann) - Mixed inflammatory infiltrate of lymphs/plasma cells/neutrophils - Multinucleated giant cells
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10
Q

Renal malakoplakia: What organisms are involved and what is the pathogenesis:

A
  • Gram negative bacteria (E. coli, proteus, klebsiella, pseudomonas, shigella, enterobacter( - Defective macrophage lysosomal digestion of phagocytosed bacteria, possible due to decreased levels of intacellular cyclic guanosine monophosphate - Inadequate elimination leading to accumulation of partially digested bacteria with deposition of calcium/iron
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11
Q

Renal malakoplakia: What are the ultrastructural features?

A
  • Bacilliform microorganisms, eithe rintact or in different stages of disintegration within phagolysosomes in macrophages
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12
Q

What genetic syndrome is associated with multiple chromophobe renal cell carcinomas?

A
  • Birt-Hogg-Dube syndrome
  • Other associations: cutaneous fibrofolliculomas, pulmonary cysts, multifocal renal tumors INCLUDING chromophobe RCC, oncocytoma, clear cell RCC, hybrid tumors
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13
Q

What are the genetic changes associated with chromophobe RCC? What is the prognosis?

A
  • Losses of whole chromosomes, including Y, 1, 2, 6, 10, 13, 17, 21
  • Better than clear cell RCC (especiallly at lower stage)
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14
Q

Name 3 histologic features, 5 IHC that can distinguish between chromophobe and clear cell RCC (eos. variant).

A
  • Histo: chromophobe has large, polygonal cells with thick plant-like borders, finely vesicular cytoplasm, with 2nd population of smaller eosinophilic cells with perinuclear clearing; randomly arranged broad fibrous septae with medium-caliber blood vessels (clear cell lacks the thick walls, has small delicate sinusoidal blood vessels)
  • IHC: CHROMOPHOBE: RCC neg, CK7+, CD10 neg, vimentin neg, e-cadherin +
  • CLEAR CELL RCC: RCC +, CK 7 neg, CD10 +, vimentin +, e-cadherin neg
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15
Q

Compare chromophobe RCC to oncocytoma with regards to nuclear features, histochemistry, EM and IHC.

A
  • chromophobe: wrinkled nuclei with perinucelar halos, binucleation; DIFFUSE staining with colloidal iron, EM: microvesicles, CK7+, BER-EP4 positive, PaX 2 negative, CK20 negative, CD15 negative
  • oncotyoma: round nuclei with prominent nucleoli; negative for colloidal iron, EM: dilated mitochondria, CK7 negative, Ber-EP4 negative, PAX2 positive, maybe CK20 positive, CD15 positive
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16
Q

Chromophobe renal cell carcinoma: List 4 histologic features

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A
  1. cell arranged in solid sheets, may have tubulocystic architecture
  2. Radomly intersecting fibrous septa with medium sized blood vessels
  3. Nuclei: irregular and hyperchromatic, wrinkled contours +//- multinucleation
  4. 2 cell types: chromophobe cell, large polygonal with plant-like cell wall borders, finely vesicular cytoplasm and eosinophilic cell which is smaller, less cytoplasm and has perinuclear halo
17
Q

What is the cell of origin in angiomyolipoma? What IHC stains are positive (5)?

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A
  • Perivascular epithelioid cell
  • IHC: vimentin, muscle-specific actin, SMA, HMB45, Melan-A
18
Q

Name a clinical syndrome associated with angiomyolipoma and 3 histological components.

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A
  • Tuberous sclerosis: AD tumor suppression gene syndrome, associated with cerebral cortical tubers, cardiac rhabdomyomas, facial angiofibromas, renal AML, lymphangioleiomyomas
  1. Smooth muscle cells: colar around blood vessels, may be perpendicular
  2. adipocytes
  3. dysmorphic blood vessels (thick-walled artery-like without elastic)
19
Q

Rhabdoid tumor of kidney: List 3 clinical features, prognosis, 3 histologic features, IHC, EM

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A

Clinical: infants <24 months, hematuria, advanced disease at presenation

Prognosis: Extremely poor, <25% survival at 1 yr

Histologic features: sheets of large cells with vesicular nuclei, eosinophilic cytoplasmic inclusions, infiltrative tumor borders

IHC: vimentin strongly positive, focal EMA and cytokeratin

EM: inclusions formed by whorled intermediate filaments

20
Q

List 4 entities in the DDx of rhabdoid tumor of kidney

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A

Nephroblastoma, neuroblastoma, mesoblastic nephroma, clear cell sarcoma of kidney

21
Q

Classify cystic diseases of the kidney

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A
  • Multicystic renal dysplasia
  • Polycystic kidney disease (AD: adult, AR: children)
  • Medullary cystic disease (medullary sponge, nephronopthisis)
  • Acquired cystic disease
  • Localized renal cysts (simple cysts)
  • Renal cysts in hereditary malformation syndromes (TSC)
  • Glomerulocystic disease
  • Extraparenchymal renal cysts (pyelocalyceal, hilar lymphangitic cysts)
22
Q

Name 4 common extrarenal manifestations of AD polycystic kidney disease

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A
  • Cysts in liver (polycystic liver disease)
  • Cysts in spleen, pancreas, lung
  • Intracranial berry aneurysms (circle of Willis)
  • Mitral valve prolapse
23
Q

Name the causes and morphology of multicystic renal dysplasia

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A

Cuases: uteropelvic obstruction, ureteral agenesis/atresia, anomalies of lower genitourinary tract

Morphology: gross: enlarged, irregular, multiple cysts of varying sizes

micro: presents of undifferentiated mesenchyme, often with cartilage and immature collecting ducts

24
Q

RCC: Name 5 risk factor and 5 associated syndromes

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A

Risk factors: smoking, obesity, hypertension, unoposed estrogen, asbestos/petrolium/heavy metals, chronic renal failure, acquired cystic disease

syndromes: TSC, VHL, hereditary clear cell carcinoma, hereditary papillary carcinoma

25
List 5 paraneoplastic syndromes associated with renal cell carcinoma
- Polycythemia - Hypercalcemia - Hypertension - Hepatic dysfunction - Virilization - cushing syndrome - eosinophilia - leukemoid reactions - amyloidosis
26
Outline the Fuhrman grading system for RCC
- Based on nuclear features with a x10 objective - Grade according to worst area - Incorporates nuclear size, nuclear outline, chromatin, nucleolar prominence - Grade 1: 10 um, round, no nucleoli; Grade 2: 10-15 micron, round, small nucleoli; Grade 3: 14-20 micron, ovan, open chromatin and large nucleoli; Grade 4: \>20 micron, pleomorphic, macronucleoli
27
Describe grossing a kidney and the important sections to take
- Look up history, imaging, orient and weigh specimen - Measure kidney, fat, tumor, length of ureter - Margins: renal vein, ureter, renal artery and soft tissue - Sections: 1/cm of tumor size, with areas of possible sarcomatoid diff. - Show extent of invasion: large vessels, perinephric fat, renal sinus fat (no capsule here), - Show normal kidney, adrenal gland if present
28
What features are necessary to report for RCC?
- histologic type - invasion into renal vein - invasion into renal sinus fat/perinephric fat - Fuhrman nuclear grade - lymphatic invasion - Sarcomatoid morphology and quantity - Geographic necrosis - Invasion into collecting system/ureter - Resection margin status - Assessment of background kidney
29
Describe the gross features of the main types of RCC
1. Clear cell: solitary, well circ., golden-yellow, hemorrhage, necrosis common 2. chromophobe: solitary, round, tan-light brown 3. papillary: well circumscribed, fibrous pseudocapsule, necrosis/hemorrahge, bilateral/multifocal 4. sarcomatoid areas: fish-flesh appearance
30
What is the most common genetic alteration in clear cell renal cell carcinoma?
Loss of chromosome 3p
31
Renal papillary adenoma: presentation, histologic features, cut-off for malignancy
Presentation: often incidental, commonly found at autopsy (7-22%) Gross features: usually less than 0.5 cm, within cortex as pale yellow-gray, well circumscribed nodule micro: complex, branching papillomatous structures with numerous complex fronds. may form tubules, glands, cords, sheets. Cells are cuboidal/polygonal with regular small nuclei and no atypia Malignant cut-off: 3 cm, also show same cytogenetic features as papillary rcc (trisomy 7 and 17)
32
Angiomyolipoma: definition, presentation, pathogenesis
Defn: benign tumor consisting of blood vessels, smooth muscle, fat Presentation: present in 25-50% of patients with tuberous sclerosis (loss-of-function mutations in TSC1/TSC2 tumor suppressor genes), susceptibilty to spontaenous hemorrhage
33
Compare the **cytogenetics** and **genetics** of sporadic and hereditary clear cell rcc and papillary RCC
1. sporadic papillary: 1. Cytogenetics: trisomy 7, 16, 17 loss of Y 2. Genetics: mutated, activated MET t (X;1) PRCC oncogenes 2. Hereditary papillary: 1. Cytogenetics: trisomy 7 2. Genetics: mutated, activated MET t (X;1) 3. Sporadic/hereditary clear cell: 1. Cytogenetics: t (3;6) t (3;8) t (3;11) 2. Genetics: loss of VHL, inactivated mutated VHL, hypermethylated VHL
34
List common causes of ureteric obstruction
- Congenital anomalies: posterior urethral valves, urethral strictures, meatal stenosis, bladder neck obstruction, ureteropelvic junction narrowing, severe vesicoureteral reflux - Urinary calculi - Benign prostatic hypertrophy - Tumors (psroste, bladder, retroperitoneal, cervix, uterus) - inflammation (prostatitis, ureteritis, urethritis, retroperitoneal fibrosis) - sloughed papillae or blood clots - pregnancy - uterine prolapse, cystocele - functional disorders (neurogenic bladder)
35
Renal stones: Name 4 main types and their prevalence
1. Calcium oxalate and phosphate-70% 2. Struvite (magnesium ammonium phosphate) 15-20% 3. Uric acid 5-10% 4. Cystine 1-2%
36
Give the general pathogenesis and predisposing conditions for each type of renal stone.
* General pathogenesis: increased urinary concentration of stone's constituents, such as it exceeds their solubility. * Predisposing conditions: * Calcium oxalate: hypercalcemia, hypercalciuria (hyperparathyroidism, diffuse bone disease, sarcoidosis), hyperoxaluria, hyperuricosiura, hypocitraturia, no known * Struvite: UTI with bacteria that convert urea to ammonia (proteus, some staph) * Uric acid: hyperuricemia (gout, leukemia), hyperuricosuria, urine pH below 5.5 * cystine: genetic defects in reabsorption of amino acids including cystine
37
What is the differential diagnosis for spindle-cell tumors of the kidney, and what IHC stains are helpful
* RCC, sarcomatoid component (AE1/AE3+, Cam 5.2+, EMA/MUC1+, desmin -, SMA -, Cd99-, S100-, MelanA-, HMB45-, MiTF neg, CK7-, CAIX-positive * Mucinous tubular and spindle cell carcinoma (AE1/AE3+, CAM 5.2+, EMA/MUC1+, neg for sarcoma/AML markers, CK7+, CAIX negative * Sarcoma (focal keratin/ema pos, postitive for desmin/actin in leiyomyosarc, CD99 in synovial sarc, neg. for AML markers, mitF, CK7) * Angiomyolipoma (cytokeratins negative, positive for esmin, actin, S100, melan A, HMB45, MiTF)

Kidney (2 decks)

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