R7 Renal Tumors, Cysts and Stones

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Author:
jknell
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205801
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R7 Renal Tumors, Cysts and Stones
Updated:
2013-03-09 12:38:42
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Renal II
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renal tumors, cysts and stones
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  1. Renal tumors, cysts and stones
    • Tumors
    • Congenital anomalies
    • Cysts
    • Hydronephrosis
    • Urinary tract obstruction
    • Stones
  2. Wilms tumor
    Genetics, etiology
    Most common kidney tumor in children <10 years old; average age 3yrs 

    • -Familial
    • -Sporadic

    • -Deletion of short arm of chromosome 11
    • -Loss of supressor gene WT-1
  3. Wilms' tumor
    • WT-1 gene, chromosome 11
    • Large, solitary well circumscribed mass
    • Immature nephroblastic elements
    • Epithelium, cartilage, bone, muscle, fibrous tissue
    • Tx: radiation chemotherapy and surgery
  4. Malformations and neoplasia
    • WAGR syndrome
    • Denys-Drash syndrome
    • Beckwith-Wiedeman
  5. WAGR syndrome
    • Wilms tumor
    • Aniridia - lack of irises 
    • Genital anomalies
    • Menatl and motor Retardation
  6. Denys-Drash syndrome
    gonadal dysgenesis and nephropathy
  7. Beckwith-Wiedenman Syndrome
    Organomegaly and cytomegaly
  8. Benign renal cell tumors I
    • Cortical adenoma
    • Medullary fibroma
    • Oncocytoma
  9. Oncocytoma
    • 5-15% of tumors
    • slow growing
    • tan brown tumors up to 12cm
    • Eosinophilic cells filled with mitochondria (red)
    • Can be found in endocrine glands (pituitary...)
    • Familial or isolated cases; solitary or multicentric
    • Always benign
  10. Renal tumors
    • Angiolyomyofibroma
    • Renal adenoma
    • Renal fibroma
    • Renal oncocytoma
  11. Angiomyofibroma
    • Tendency to hemorrhage, epilepsy, skin lesions, mental retardation
    • TSC 1 or TSC 2 tumor suppressor gene may be defective
  12. Renal adenoma
    May grow and behave like renal cell carcinoma
  13. Renal fibroma
    totally harmless
  14. Renal cell carcinoma
    Epidemiology
    • RCC represents up to 3% of all newly diagnosed primary cancers
    • 85% of all renal tumors are RCC
    • 12,000 deaths from RCC; 30,000 new cases every year
    • 11th major cause of cancer
  15. RCC
    Risk factors
    • Tobacco: including pipes, cigars and snuff
    • Obesity
    • Hypertension
    • Estrogen treatment
    • Asbestos exposure
    • Petroleum products
    • Heavy metals (cadmium, mercury, lead)
    • polycystic disease/dialysis
    • Von Hippel Lindaue disease: hemangioblastoma, retina, kidney and cerebellum
    • VHL gene
    • Men > women (based on risk factors)
  16. VHL
    • VHL tumor suppressor gene
    • -controls ubiquitin ligase which targets certain proteins for destruction

    VHL mutations → HIF-1 is high and proangiogenic proteins (VEGF, PDGF, TGF alpha and beta, IGF)... increase their activity and cells and vessels proliferate

    VHL mutations increase likely-hood of tumor or cysts
  17. VHL gene in renal cancer
    VHL gene: signal transduction/cell adhesion, ubiquitin ligase, involvement in protein degradation

    -Chromosome 3p25-26

    -Tumor associated with inherited mutations

    -Hemangioblastomas

    -Angiomas and cysts of viscera
  18. VHL gene in renal cancer
    "clear cell" appearance
    • Clear cell carcinoma
    • -70-80% of renal cancers
    • -Associated with VHL gene
  19. Renal cell carcinoma
    other types
    • Papillary renal cell ca
    • -10-15%
    • -Associated with MET protooncogene

    • Chromophobe renal cell ca
    • -5% (includes oncocytomas)
  20. RCC
    Presentation, sx
    • Triad: (all 3 present in only 10%)
    • 1. Pain
    • 2. Hematuria
    • 3. Flank mass

    • -Hematuria only 90%
    • -Sx more likeloy when tumor is near renal pelvis
    • -Many tumors located at upper pole of kidney
    • -Men>women (based on risk factors)
    • -Mid 30s to late middle age
  21. RCC
    endocrine effects
    • Paraneoplastic effects of RCC
    • -Polycythemia - erythropoietin
    • -HTN - renin
    • -Hypercalcuria - PTH
    • -Feminization/masculinization - gonadotrophin
    • -Cushing's syndrome - steroids
  22. RCC survival and mets
    • 5 yr survival 45%
    • without mets 70%
    • mets to lungs...50%
    • mets to bone... 30%
    • mets to kidney... 10%
  23. Non-malignant tumors II
    • Renal fibroma
    • Angiomyolipoma
  24. Renal fibroma
    • Common, harmless
    • Usually found at autopsy
    • Harmartoma
  25. Angiomyolipoma
    • Found in patients with tuberous sclerosis
    • -25-50% of whom get this tumor

    Associated with seizures and skin lesions
  26. Cancer of the ureter
    • Transitional cell carcinoma
    • -uncommon tumor
    • -high mortality
    • -Presentation: painless hematuria, hydronephrosis
    • -5yr survival <10%
    • -Risk factors: analgesic uses (all urothelial tumors)
  27. Bladder cancer
    Presentation, pathogenesis, Tx/prognosis
    • Transitional epithelium
    • papilloma

    • Presentation:
    • -asymptomatic until...
    • -painless hematuria
    • -Male>female 3:1

    • Pathogenesis:
    • -tobacco products
    • -chronic cystitis
    • -Schistosomiasis (Nile delta)
    • -Cyclophosphamide (cancer therapeutic)
    • -B-naphthylamine (used in moth balls)

    • Tx/prognosis:
    • -Surgical removal (part or entire organ)
    • -Depth of invasion determines outcome:
    • -5yr survival 57%
    • -Invasive ca <20%
    • Polycystic renal disease
    • -Bilateral expanding masses, destroying renal parenchyma
  28. PCKD
    genetics, epidemiology
    • Epidemiology:
    • -1/1000 persons affected
    • -APKD accounts for 10% of renal failure cases
    • -APKD → HTN, urinary infections


    • Genetics:
    • -APKD-1 is involved in most cases (chromosome 16)
    • -APKD-2 is involved in 10% (chromosome 4)
    • -Polycystin gene: lectin-like and fibronectin-like domain... abnl development of ECM by tubules leads to cyst formation
  29. Polycystic disease
    presentation
    • 10-30% of APKD pts get cerebral aneurism with high rate of subarachnoid hemorrhage
    • Hypertension, urinary infections
    • Painful rupture of cysts → flank pain and hematuria

    • Sx present in 4th decade
    • Renal failure by age 50
  30. Kidney stones
    chemical compositions
    • Calcium oxalate ± phosphate
    • -75%

    • Magnesium ammonium phosphate
    • -15%

    • Uric acid or cystine
    • -10%
  31. Kidney stones
    risk factors
    • Lack of portable water
    • hot climate
  32. Calcium oxalate
    Causes
    • Causes:
    • -Excessive absorption of calcium
    • -Hypercalcuria
    • -Defective renal absorption
    • -Hyperparathyroidism
    • -Vitamin D intoxication
    • -High meat diet; vegetable diet somewhat protective
  33. Mg/NH3/PO4 stones
    • Associated with urinary tract infections
    • -Proteus (and other urea splitting bacteria)
    • -Staphylococci
    • -Bacteria nidi
  34. Inhibitors of stone formation
    • Tamm Horsfal protein
    • Crystal matrix protein
    • Nephrocalcin
    • Uroprontin/osteopontin
    • Alpha-1-antitrypsin
    • Glycosaminoglycans
    • Citrate
    • Pyrophosphate
    • Zinc/Magnesium
  35. mechanism of stone formation
  36. Urolithiasis
    epidemiology
    • Mainly affects kidney, but ureter and bladder are possible
    • Kidney stones found in 1% of all autopsies
    • ♂ > ♀ symptomatic
    • Familial factors
  37. Acquired hydronephrosis
    Obstruction in the urinary pathway leads to retrograde fluid accumulation  with distention of renal calyces

    • Risk factors/causes:
    • -Pregnancy
    • -Tumors (prostate, uterus)
    • -Neurogenic bladder
    • -Infection (prostatitis)
    • -Obstruction (necrotic papilla, stone
  38. Congenital hydronephrosis
    Causes
    • Atresia of ureter
    • Ureteral valves
    • Aberrant renal artery compressing ureter
    • Renal ptosis with torsion
    • Kinking of ureter
  39. Congenital malformations
    • -Fetal lobulation - most are benign; lobulations will disappear as child develops

    • -Polycystic renal disease

    • -Agenesis (if unilateral, the contralateral kidney will be abnormally large to compensate)
    • -Hypoplasia
    • -Horseshoe kidney
    • -Cysts of the medulla
    • -Simple cysts
    • Multiple renal arteries and aberrant ureter
    • **Ureter is vulnerable to abdomen causing compression
    • Horsehoe kidney
    • Ureter pass over lower poll of the kidney
    • Inferior Mesenteric Artery 'traps' kidneys in base of abdomen/pelvis
  40. Hematopoietic tumors
    Kidney is common site for metastasis for lymphoma


    • Multiple myeloma (Bence Jones proteins are toxic  to tubular epithelium)
    • -Renal insufficiency
    • -Infection common
    • -Amyloidosis in 10%

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