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Flashcards

Renal cell carcinoma and Wilms tumor

Shylla T Inglês 61 flashcards

Flashcards do conjunto (61)

  1. 1How does polycystic kidney disease differ from kidneys on dialysis in end stage renal disease?Polycystic: big cystic kidneys
    Kidneys on dialysis: small shrunken kidneys
  2. 2What are the processes that cause renal osteodystrophy?Damage to bone occurring due to renal failure
    1. Osteitis fibrosa cystica (hyperparathyroidism burns out bone)
    2. Osteomalacia
    3. Osteoporosis
  3. 3AngiomyolipomaHamartoma comprised of blood vessels, smooth muscle, and adipose tissue
  4. 4What renal disease is tuberous sclerosis associated with?angiomyolipoma
  5. 5Renal cell carcinomaMalignant epithelial tumor arising from kidney tubules
  6. 6Triad of renal cell carcinomaHematuria, flank pain, palpable mass (rarely have all three)
  7. 7What is the most common single presenting symptom of renal cell carcinoma?Hematuria
  8. 8Presentation of renal cell carcinomaHematuria, flank pain, palpable mass
    Fever, weight loss, paraneoplastic syndrome
    (EPO causing --> reactive polycythemia
    renin causing --> HTN
    PTHrP causing --> hypercalcemia
    ACTH causing --> Cushing's)
  9. 9What side can renal cell carcinoma rarely cause a varicocele on?Left side (due to spermatic vein draining into the renal vein before draining into the IVC)
  10. 10How does a left sided varicocele present in renal cell carcinoma?Carcinoma can block the renal vein, preventing drainage of left spermatic vein, which drains into the left renal vein
  11. 11What is the most common subtype of renal cell carcinomaClear cell
  12. 12Gross exam of renal cell carcinomaYellow mass
  13. 13Gene that is lost in renal cell carcinoma and sequelae of thatVHL (3p) tumor suppressor gene lost, causing increased IGF-1 (increased growth), increased HIF transcription factor (which increases PDGF and VEGF to promote angiogenesis and tumor growth)
    loss of VHL TSG --> 1) increased IGF-1 (increases growth), and 2) increased HIF TF --> increases PDGF and VEGF --> increased angiogenesis and tumor growth
  14. 14What are the two ways renal cell carcinoma can form?Sporadic or hereditary
  15. 15What is the biggest risk factor for a sporadic renal cell carcinoma tumor?Smoking
  16. 16Sporadic renal cell carcinoma tumorCommonly arise in adult males around 60
    Single tumor in the upper left pole of the kidney
    Major risk factor is smoking
  17. 17Where do sporadic renal cell carcinoma tumors generally arise?Upper left pole of a single kidney
  18. 18Who do hereditary renal cell carcinoma tumors generally arise in?Younger adults
  19. 19Where are hereditary renal cell carcinoma tumors in the kidney?Bilateral
  20. 20Von Hippel-Lindau DiseaseAuto dom disorder associated with inactivation of VHL gene leading to increased risk for hemangioblastoma of the cerebellum and renal cell carcinoma
  21. 21Two tumors associated with Von Hippel-Lindau DiseaseHemangioblastoma of the cerebellum
    Renal cell carcinoma
  22. 22Where does renal cell carcinoma like to grow?Into the renal vein
  23. 23What nodes does renal cell carcinoma like to spread to?Retroperitoneal lymph nodes
  24. 24Staging of renal cell carcinomaBased on Tumor size (likes to grow into the renal veins) and Nodes (likes to spread to the retroperitoneal nodes)
  25. 25What comprises most of a Wilms Tumor?Blastema
  26. 26What is blastema?Immature kidney mesenchyme that makes up much of Wilms Tumor
  27. 27What is Wilm's tumor comprised of?blastema, primitive glomeruli and tubules, and stromal cells
  28. 28What is the most common malignant renal tumor in children?Wilms Tumor (average age is 3 years)
  29. 29What is the average age of Wilms Tumor?3 years
  30. 30Presentation of Wilms TumorLarge unilateral flank mass with hematuria and hypertension due to renin secretion
  31. 31What causes hypertension in Wilms TumorRenin secretion
  32. 32What is the cause of 90% of Wilms TumorsSporadic
  33. 33Name three syndromes Wilms Tumors can be associated with1. WAGR Syndrome
    2. Denys-Drash Syndrome
    3. Beckwith-Wiedemann Syndrome
  34. 34WAGR Syndrome Overview-Associated with Wilms Tumor
    -Wilms tumor, Aniridia (absence of iris), Genital abnormalities, mental and motor Retardation
    Associated with deletion of WT1 tumor suppressor gene located at 11p13
  35. 35Denys-Drash Syndrome-Associated with Wilms Tumor
    -Progressive renal (glomerular) disease, male pseudohermaphroditism
    -Associated with mutations of WT1
  36. 36Beckwith-Wiedemann Syndrome-Associated with Wilms Tumor
    -Neonatal hypoglycemia, muscular hemihypertrophy, organomegaly (tongue included)
    -Associated with mutation in WT2 gene cluster (imprinted at 11p15.5), particularly IGF-2
  37. 37Polyuria, renal tubular acidosis type II, growth failure, electrolyte imbalances, hypophosphatemic ricketsFanconi syndrome (multiple combined dysfunction of the proximal convoluted tubule)
  38. 38Athlete with polycythemia2° to erythropoietin injection
  39. 39Periorbital and/or peripheral edema, proteinuria (> 3.5g/day), hypoalbuminemia, hypercholesterolemiaNephrotic syndrome
  40. 40Hereditary nephritis, sensorineural hearing loss, retinopathy, lens dislocationAlport syndrome (mutation in collagen IV)
  41. 41“Waxy” casts with very low urine flowChronic end-stage renal disease
  42. 42Nodular hyaline deposits in glomeruliKimmelstiel-Wilson nodules (diabetic nephropathy)
  43. 43Podocyte fusion or “effacement” on electron microscopyMinimal change disease (child with nephrotic syndrome)
  44. 44Spikes” on basement membrane, “dome-like” subepithelial depositsMembranous nephropathy (nephrotic syndrome)
  45. 45RBC casts in urineGlomerulonephritis
  46. 46“Tram-track” appearance of capillary loops of glomerular basement membranes on light microscopyMembranoproliferative glomerulonephritis
  47. 47Anti–glomerular basement membrane antibodiesGoodpasture syndrome (glomerulonephritis and hemoptysis)
  48. 48Cellular crescents in Bowman capsuleRapidly progressive (crescentic) glomerulonephritis
  49. 49“Wire loop” glomerular capillary appearance on light microscopyDiffuse proliferative glomerulonephritis (usually seen with lupus)
  50. 50Linear appearance of IgG deposition on glomerular and alveolar basement membranesGoodpasture syndrome
  51. 51“Lumpy bumpy” appearance of glomeruli on immunofluorescencePoststreptococcal glomerulonephritis (due to deposition of IgG, IgM, and C3)
  52. 52Necrotizing vasculitis (lungs) and necrotizing glomerulonephritisGranulomatosis with polyangiitis (Wegener; PR3-ANCA/c-ANCA) and Goodpasture syndrome (anti–basement membrane antibodies)
  53. 53Thyroid-like appearance of kidneyChronic pyelonephritis (usually due to recurrent infections)
  54. 54WBC casts in urineAcute pyelonephritis
  55. 55Renal epithelial casts in urineIntrinsic renal failure (eg, ischemia or toxic injury)
  56. 56Hyperaldosteronism txSpironolactone
  57. 57Nephrotic syndrome (adults)Membranous nephropathy
  58. 58Nephrotic syndrome (kids)Minimal change disease
  59. 59Glomerulonephritis (adults)Berger disease (IgA nephropathy
  60. 60Kidney stonesCalcium = radiopaque
    Struvite (ammonium) = radiopaque (formed by urease; ⊕ organisms such as Klebsiella, Proteus species, and S saprophyticus)
    Uric acid = radiolucent
    Cystine = faintly radiopaque
  61. 61Renal tumorRenal cell carcinoma: associated with von Hippel-Lindau and cigarette smoking; paraneoplastic syndromes (EPO, renin, PTHrP, ACTH)

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FlashCards sobre Sem título, criado por Shylla T em 13-06-2021.
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Shylla T
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