Renal Pathology 2.ppt

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Renal Pathology 2.ppt

  1. 1. <ul><li>Diseases of Blood Vessels </li></ul><ul><li>Nearly all diseases of kidney involve blood </li></ul><ul><li>vessels. </li></ul><ul><li>Kidneys involved in pathogenesis of essential </li></ul><ul><li>and secondary hypertension </li></ul><ul><li>Systemic vascular disease (i.e. arteritis) also </li></ul><ul><li>involve kidney </li></ul>
  2. 2. <ul><li>Benign Nephrosclerosis </li></ul><ul><li>Sclerosis of renal arterioles and small </li></ul><ul><li>arteries </li></ul><ul><li>a) some degree of ischemia </li></ul><ul><li>i) hypertension </li></ul><ul><li>Renal changes associated with benign </li></ul><ul><li>hypertension </li></ul><ul><li>a) always associated with hyaline arteriosclerosis </li></ul><ul><li>i) deposits in arterioles </li></ul><ul><li>- extravasation of plasma proteins through injured endothelial cells; also in BM </li></ul>
  3. 3. <ul><li>ii) results in narrowing of lumen </li></ul><ul><li>no renal insufficiency nor uremia in </li></ul><ul><li>uncomplicated cases </li></ul><ul><li>a) diabetes, blacks, more severe blood pressure elevations  risk of renal insufficiency </li></ul><ul><li>Kidneys are atrophic </li></ul><ul><li>Many renal diseases cause hypertension which </li></ul><ul><li>in turn may lead to benign nephrosclerosis. </li></ul><ul><li>Therefore this disease seen simultaneously </li></ul><ul><li>with other diseases of kidney </li></ul>
  4. 4. <ul><li>This disease by itself usually does not cause </li></ul><ul><li>severe damage </li></ul><ul><li>a) mild oliguria </li></ul><ul><li>b) loss (slight) of concentrating mechanism </li></ul><ul><li>c) decreases GFR </li></ul><ul><li>d) mild degree of proteinuria is a constant finding </li></ul><ul><li>These patients usually die from hypertensive </li></ul><ul><li>heart disease or cerebrovascular disease rather </li></ul><ul><li>than from renal disease </li></ul><ul><li>Pathogenesis </li></ul><ul><li>a) medial and intimal thickening. Due to: </li></ul><ul><li>i) age, hemodynamic changes, genetic factors, etc. </li></ul>
  5. 5. <ul><li>Malignant hypertension </li></ul><ul><li>(malignant nephrosclerosis) </li></ul><ul><li>malignant nephrosclerosis is the f orm of renal </li></ul><ul><li>disease associated with malignant hypertension </li></ul><ul><li>Less common than benign </li></ul><ul><li>May arise de novo (without preexisting </li></ul><ul><li>hypertension) or may arise suddenly in patient with mild hypertension (i.e., essential benign hypertension) </li></ul><ul><li>Frequent cause of death from uremia </li></ul>
  6. 6. <ul><li>Factors: </li></ul><ul><li>a) initial event – some form of vascular </li></ul><ul><li>damage to kidney </li></ul><ul><li>b) result is increased permeability of small blood vessels to fibrinogen and other plasma proteins, endothelial injury </li></ul><ul><li> and platelet deposits </li></ul><ul><li>c) This leads to appearance of fibrinoid necrosis in small arteries and arterioles and intravascular thrombosis </li></ul>
  7. 7. d) platelets (platelet derived growth factors) and plasma cause intimal hyperplasia of vessels resulting in hyperplastic arteriosclerosis, which is typical of malignant hypertension e) narrowing of renal afferent arteriole (i.e., progressive ischemia) stimulates angiotensin II production (ischemic- induced) with renin secretion increased. Aldosterone also increased. i) self perpetuating cycle - constriction (Angio II)  vasoconstriction  ischemia  renin  etc.
  8. 8. <ul><li>Diastolic pressure > 130 mmHg, papilledema, </li></ul><ul><li>encephalopathy, CV disorders, renal failure </li></ul><ul><li>a) often, early symptoms due to  intracranial pressure </li></ul><ul><li>i) headache, nausea, vomiting, visual impairments </li></ul><ul><li>b) hypertensive crisis </li></ul><ul><li>i) loss of consciousness, convulsions </li></ul><ul><li>ii) proteinuria and hematuria (micro- or macro-) </li></ul><ul><li>90% deaths due to uremia </li></ul><ul><li>10% deaths due to CV or cerebral disorders </li></ul><ul><li>(hemorrhage) </li></ul>
  9. 9. This is a different kind of arteriosclerosis. This is hyperplastic arteriolosclerosis, which most often appears in the kidney in patients with malignant hypertension. The arteriolar wall is markedly thickened and the lumen is narrowed. Arteriosclerosis, or &quot;hardening of the arteries&quot; is a generic term that includes atherosclerosis, arteriolosclerosis, and medial calcific sclerosis.
  10. 10. <ul><li>Renal Artery Stenosis </li></ul><ul><li>Unilateral is uncommon cause of hypertension </li></ul><ul><ul><li>a) ~ 70 % due to atheromatous plaque at origin of renal artery </li></ul></ul><ul><ul><li>i) second leading cause is fibromuscular dysplasia of renal artery (hyperplasia of all layers) </li></ul></ul><ul><li>- More common in women </li></ul><ul><li>- Tend to occur in younger groups (20-30 yrs) </li></ul>
  11. 11. <ul><li>b) potentially curable form of hypertension </li></ul><ul><li>i) related to degree of stenosis </li></ul><ul><li>- caused primarily by renin secretion </li></ul><ul><li>- ACE inhibitors show marked  in arterial blood pressure </li></ul><ul><li>- revascularization </li></ul><ul><li>Ischemic kidney shows signs of diffuse ischemic </li></ul><ul><li>atrophy </li></ul><ul><li>Patients present usually resembling essential </li></ul><ul><li>hypertension </li></ul><ul><li>arteriography required to localize stenotic lesion </li></ul><ul><li>70-80 % cure rate </li></ul>
  12. 12. <ul><li>Thrombotic Microangiopathies </li></ul><ul><li>Clinical syndromes </li></ul><ul><li>Widespread thrombosis in microcirculation (a/c) </li></ul><ul><li>Damage to endothelial cells !! </li></ul><ul><li>Diseases: </li></ul><ul><li>a) childhood hemolytic-uremia syndrome </li></ul><ul><li>(HUS) </li></ul><ul><li>b) Thrombotic thrombocytopenic purpura </li></ul><ul><li>Most follow intestinal infection ( E. coli ) </li></ul><ul><li>Disease is one of main causes of acute renal </li></ul><ul><li>failure in children </li></ul><ul><li>Vasoconstriction (decreased NO, increased </li></ul><ul><li>endothelin-1, decreased PGI 2 ) </li></ul>
  13. 13. <ul><li>Although the various diseases have diverse </li></ul><ul><li>etiologies, 2 predominant factors  </li></ul><ul><li>a) endothelial injury and activation, leading to vascular thrombosis and, </li></ul><ul><li>b) platelet aggregation </li></ul><ul><li>c) both of these causing vascular obstruction and vasoconstriction </li></ul><ul><li>1.- Endothelial injury </li></ul><ul><li>activation can be initiated by a variety of </li></ul><ul><li>agents, while some remain elusive </li></ul><ul><li>a) denuding the endothelial cells, exposes vascular to thrombogenic subendothelium </li></ul><ul><li>i)  NO, PGI 2 , enhance platelet aggregation and vasoconstriction </li></ul>
  14. 14. <ul><li>ii) vasoconstriction also initiated via endothelial derived endothelin-1 </li></ul><ul><li>iii) activation of endothelial cells increases adhesiveness to platelets, etc. </li></ul><ul><li>iv) endothelial cells elaborate large multimers of vW factor  platelet aggregation </li></ul><ul><li>2.- Platelet Aggregation </li></ul><ul><li>serum factors causing platelet aggregation </li></ul><ul><li>a) large multimers of vW factor (secreted by endothelial cells) </li></ul><ul><li>i) usually cleaved by ADAMTS-13 (vW factor-cleaving metalloprotease) </li></ul>
  15. 15. <ul><li>HUS/TTP </li></ul><ul><li>1.- Classic childhood HUS (> 75% following infection) </li></ul><ul><li>bloody diarrhea  intestinal infection </li></ul><ul><li>a) verocytotoxin-releasing bacteria </li></ul><ul><li>i) Verocytotoxin-producing strains of E. coli (eg 0157:H7 or 0103); </li></ul><ul><li>ii) Similar to Shigella toxin. </li></ul><ul><li>iii) undercooked hamburger </li></ul><ul><li>iv) “petting” zoos </li></ul>
  16. 16. <ul><li>characterized: </li></ul><ul><li>a) sudden onset (post GI or influenza infection) </li></ul><ul><li>b) hematemesis </li></ul><ul><li>c) melena </li></ul><ul><li>d) severe oliguria </li></ul><ul><li>e) hematuria </li></ul><ul><li>f) hemolytic anemia (microangiopathic) </li></ul><ul><li>g) hypertension in > 50% of cases </li></ul>
  17. 17. <ul><li>Pathogenesis </li></ul><ul><li>a) related to Shigella toxin </li></ul><ul><li>i) affects endothelium </li></ul><ul><li>-  adhesion of leukocytes </li></ul><ul><li>-  endothelin and  NO </li></ul><ul><li>- endothelial lysis (in presence of cytokines such as TNF) </li></ul><ul><li>ii) these changes favor thrombosis and vasoconstriction </li></ul><ul><li>iii) verocytotoxin can directly bind to platelets and cause activation </li></ul><ul><li>most patients recover in few weeks, with </li></ul><ul><li>proper care (i.e., dialysis, etc); < 5% lethality </li></ul>
  18. 18. <ul><li>2.- Adult HUS </li></ul><ul><li>associated with: </li></ul><ul><li>a) infection </li></ul><ul><li>i) typhoid fever </li></ul><ul><li>ii) E. coli septicemia </li></ul><ul><li>iii) etx or shiga toxin </li></ul><ul><li>iv) viral infections </li></ul><ul><li>b) antiphospholipid syndrome </li></ul><ul><li>i) SLE </li></ul><ul><li>ii) similar to membranoproliferative GN but w/out immune complex deposits </li></ul><ul><li>c) complication of pregnancy (“postpartum renal failure” </li></ul>
  19. 19. <ul><li>d) vascular renal disease </li></ul><ul><li>i) systemic sclerosis </li></ul><ul><li>ii) malignant hypertension </li></ul><ul><li>e) chemotherapeutic and immunosuppressive drugs </li></ul><ul><li>i) mitomycin </li></ul><ul><li>ii) cyclosporine </li></ul><ul><li>iii) bleomycin </li></ul><ul><li>iv) cisplatin </li></ul><ul><li>v) radiation Tx </li></ul><ul><li>3.- Familial HUS </li></ul><ul><li>recurrent thromboses (~ 50 lethality) </li></ul><ul><li>deficit of complement regulatory protein </li></ul><ul><li>a) Factor H </li></ul>
  20. 20. <ul><li>4.- Idiopathic Thrombotic Thrombocytopenic Purpura </li></ul><ul><li>Manifested by: </li></ul><ul><li>a) thrombi in glomeruli </li></ul><ul><li>b) fever </li></ul><ul><li>c) hemolytic anemia </li></ul><ul><li>d) neurologic symptoms </li></ul><ul><li>e) thrombocytopenic purpura </li></ul><ul><li>defect in ADAMTS-13 (acquired or inherited) </li></ul><ul><li>a) normally cleaves large vW multimers </li></ul><ul><li>i) large vW factors promote platelet aggregation </li></ul><ul><li>more common in women </li></ul><ul><li>most patients < 40 years </li></ul>
  21. 21. <ul><li>neurologic involvement is dominant feature </li></ul><ul><li>renal involvement in ~ 50% of patients </li></ul><ul><li>a) eosinophilic thrombi in glomerular capillaries, interlobular artery and afferent arterioles </li></ul><ul><li>b) similar changes as with HUS </li></ul><ul><li>exchange transfusion and steroid Tx  </li></ul><ul><li>mortality rate to < 50% </li></ul>
  22. 22. <ul><li>Other vascular disorders </li></ul><ul><li>Atherosclerotic renal disease </li></ul><ul><li>a) bilateral stenosis </li></ul><ul><li>i) fairly common in older adults </li></ul><ul><li>ii) cause of chronic ischemia </li></ul><ul><li>Atheroembolic renal disease </li></ul><ul><li>a) via atheroma in older patients with severe atherosclerosis </li></ul><ul><li>Sickle cell disease </li></ul><ul><li>a) vasa recta plugging by sickled cells </li></ul><ul><ul><li>i) hematuria </li></ul></ul><ul><ul><li>ii)  renal concentrating mechanism </li></ul></ul><ul><ul><li>iii) patchy papillary necrosis </li></ul></ul><ul><ul><li>iv) proteinuria </li></ul></ul>
  23. 23. <ul><li>Diffuse cortical necrosis </li></ul><ul><ul><li>a) uncommon </li></ul></ul><ul><ul><li>i) obstetric emergency </li></ul></ul><ul><ul><li>ii) septic shock </li></ul></ul><ul><ul><li>iii) following extensive surgery </li></ul></ul><ul><ul><li>b) glomerular and arteriolar microthrombi </li></ul></ul><ul><ul><li>c) unilateral or patchy involvement are compatible with survival </li></ul></ul><ul><li>Renal infarcts </li></ul><ul><li>a) favored sites for infarcts </li></ul><ul><li>i) “end organ” nature of vasculature </li></ul><ul><li>b) most infarcts due to emboli </li></ul><ul><li>i) via left ventricle/atria as result of MI </li></ul><ul><li>- mural thrombosis </li></ul>
  24. 24. <ul><li>Cystic Diseases </li></ul><ul><li>Common and difficult to diagnose </li></ul><ul><li>In adult polycystic disease – major cause of </li></ul><ul><li>chronic renal failure </li></ul><ul><li>Confused with malignant tumors </li></ul><ul><li>Simple cyst </li></ul><ul><li>a) Innocuous lesion </li></ul><ul><li>b) Occur as single or multiple cysts </li></ul><ul><li>c) Usually 1-5 cm diameter </li></ul><ul><li>d) Clear fluid, smooth membrane, gray </li></ul><ul><li>glistening </li></ul>
  25. 25. <ul><li>e) Single layer of cuboidal cells </li></ul><ul><li> f) Usually confined to cortex </li></ul><ul><li> g) No clinical significance </li></ul><ul><li>Importance to differentiate from tumors </li></ul><ul><li>a) are fluid filled rather than solid </li></ul><ul><li>b) have smooth contours </li></ul><ul><li>c) almost always avascular </li></ul><ul><li>Occur in patients with end-stage renal disease </li></ul><ul><li>who have undergone long term dialysis </li></ul><ul><li>Occasionally, renal adenomas or adenosarcoma </li></ul><ul><li>arise from these cysts </li></ul>
  26. 26. <ul><li>Adult polycystic kidney disease (autosomal dominant) </li></ul><ul><li>Multiple expanding cysts of both kidneys that </li></ul><ul><li>eventually destroy parenchyma of kidney </li></ul><ul><li>Accounts for 10% of chronic renal failure </li></ul><ul><li>In 90% of families, PKD1 (defective gene) is </li></ul><ul><li>located on chromosome #16 </li></ul><ul><li>a) encodes for protein (polycystin-1), extracellular and is a cell membrane associated protein </li></ul><ul><li>b) how mutations in this gene cause cysts formation is unclear </li></ul>
  27. 27. <ul><li>Polycystin 2 (PKD2 gene) mutations also cause </li></ul><ul><li>cyst formation </li></ul><ul><li>No symptoms until 4th decade </li></ul><ul><li>a) by then, kidneys are very large </li></ul><ul><li>b) common complaint is “flank pain” </li></ul><ul><li>c) hematuria </li></ul><ul><li>d) most important complications </li></ul><ul><li>i) hypertension (~75% patients) </li></ul><ul><li>ii) UTI </li></ul><ul><li>iii) aneurysms in circle of Willis (10- 30%) and risk for subarachnoid hemorrhage </li></ul>
  28. 28. <ul><li>iv) Asymptomatic liver cysts in ~30- 40% </li></ul><ul><li>v) fatal disease (uremia or hypertension) </li></ul><ul><li>vi) progresses very slowly </li></ul><ul><li>viii) Treatment with renal transplantation </li></ul><ul><li>Childhood polycystic kidney disease (autosomal recessive) </li></ul><ul><li>Rare </li></ul><ul><li>Serious manifestations at birth and young </li></ul><ul><li>infants may die quickly </li></ul><ul><li>a) pulmonary failure </li></ul><ul><li>b) renal failure </li></ul>
  29. 29. <ul><li>Numerous small cysts in cortex and medulla </li></ul><ul><li>Bilateral disease </li></ul><ul><li>Many epithelial cysts in liver </li></ul><ul><li>Patients who survive infancy develop liver </li></ul><ul><li>cirrhosis (congenital hepatic cirrhosis) </li></ul><ul><li>Unidentified gene location on chromosome 6p </li></ul><ul><li>Urinary Outflow-Obstruction </li></ul><ul><li>Renal Stones </li></ul><ul><li>Urolithiasis: Calculus formation at any level in </li></ul><ul><li>urine collecting system, most often arise in </li></ul><ul><li>kidney </li></ul>
  30. 32. <ul><li>Occur frequently (!1% of all autopsies) </li></ul><ul><li>More common in males </li></ul><ul><li>Familial tendency </li></ul><ul><li>~75% of renal stone </li></ul><ul><li>a) calcium oxalate </li></ul><ul><li>b) calcium phosphate </li></ul><ul><li>15% composed of magnesium ammonium </li></ul><ul><li>phosphate </li></ul><ul><li>10% uric acid or cystine stones </li></ul><ul><li>All stones composed of mucoprotein </li></ul>
  31. 33. <ul><li>Cause of stones is obscure </li></ul><ul><li>a) Supersaturation in urine of stones constituents (exceeds solubility) </li></ul><ul><li>b) 50% of patients forming “calcium stones” do not have increased plasma Ca ++ but do have high urine Ca ++ </li></ul><ul><li>i) most Ca ++ absorbed from gut in large amounts (absorptive hypercalciuria) </li></ul><ul><li>ii) only 5-10% has associated hypercalcemia </li></ul>
  32. 34. <ul><ul><li>- hyperparathyroidism </li></ul></ul><ul><ul><li>- Vit D intoxication </li></ul></ul><ul><ul><li>- Sarcoidosis (autoimmune disease, bacterial) </li></ul></ul><ul><li>- productions of Vit D (toxic) </li></ul><ul><li>c) Magnesium ammonium Phosphate stones </li></ul><ul><li>i) almost always occur in patients with alkaline urine due to UTI </li></ul><ul><li>ii) Proteus vulgaris and Staph split urea in kidney and therefore predispose patient to urolithiasis </li></ul>
  33. 35. <ul><li>Gout and diseases involved with rapid cell </li></ul><ul><li>turnover (e.g. leukemia) lead to high uric acid </li></ul><ul><li>levels in urine and possibility of uric acid stones </li></ul><ul><li>Unlike magnesium ammonium phosphate stone, </li></ul><ul><li>both uric acid and cystine stones are more </li></ul><ul><li>likely to form when urine is acidic (pH < 5.5) </li></ul><ul><li>Stone formation 80% unilateral </li></ul><ul><li>Hematuria and predispose to infection </li></ul>
  34. 36. <ul><li>Hydronephrosis </li></ul><ul><li>Dilation of renal pelvis and calyces with atrophy </li></ul><ul><li>of parenchyma caused by obstruction of outflow </li></ul><ul><li>of urine </li></ul><ul><li>Most common causes: </li></ul><ul><li>a) congenital </li></ul><ul><li>i) atresia of the urethra (absence of a normal body passage or opening from an organ to other parts of the body) </li></ul>
  35. 37. <ul><li>b) acquired </li></ul><ul><li>i) stones </li></ul><ul><li>ii) tumors </li></ul><ul><li>iii) inflammation </li></ul><ul><li>iv) spinal cord damage with paralysis of bladder </li></ul><ul><li>v) normal pregnancy </li></ul><ul><li>Bilateral only if blocked below level of ureters </li></ul><ul><li>Major problems are tubular with impaired </li></ul><ul><li>concentration mechanisms </li></ul><ul><li>Obstruction leads to inflammatory response a) interstitial fibrosis </li></ul><ul><li>Complicating pyelonephritis is common </li></ul><ul><li>Pain from distension of collecting system </li></ul>
  36. 38. <ul><li>Tumors </li></ul><ul><li>Most common malignant tumor is: </li></ul><ul><li>a) renal cell carcinoma (80-85% of all 1° Ca in kidney) </li></ul><ul><li>b) nephroblastoma (Wilms tumor) </li></ul><ul><li>c) urothelial tumors of calyces and pelvis </li></ul><ul><li>Tumor of lower urinary tract are 2x as common </li></ul><ul><li>as renal cell Cancer </li></ul>
  37. 39. <ul><li>Benign tumors </li></ul><ul><li>Renal papillary adenoma </li></ul><ul><li>a) arising tubular epithelium </li></ul><ul><li>b) common (7-22 % found on autopsy) </li></ul><ul><li>Renal fibroma or harmartoma (renomedullary </li></ul><ul><li>interstitial cell tumor) </li></ul><ul><ul><li>a) firm white-gray, < 1cm, found in pyramids (fibroblast-like cells) </li></ul></ul><ul><li>Oncocytoma (large nucleoli); eosinophilic cells </li></ul>
  38. 40. <ul><li>Malignant Tumors </li></ul><ul><li>Renal cell Ca (adenocarcinoma of kidney) </li></ul><ul><li>Derived from renal tubular epithelial cells </li></ul><ul><li>a) located primarily in cortex </li></ul><ul><li>2-3% of all cell Ca in adults (~30,000 cases/yr) </li></ul><ul><li>6th to 7th decades in life </li></ul><ul><li>Male preponderance (~ 3:1) </li></ul>
  39. 41. <ul><li>Higher risk in smokers (2:1) and occupational </li></ul><ul><li>exposure to cadmium, hypertension and acute renal failure and acquired cystic disease. </li></ul><ul><li>30 fold increase in susceptibility in patients with </li></ul><ul><li>polycystic disease </li></ul><ul><li>Classification: </li></ul><ul><li>1. clear cell Cancer </li></ul><ul><li>i) most common (~ 85 % renal ca) </li></ul><ul><li>a) most are sporadic (~ 95%), nonpapillary </li></ul><ul><li>b) familial links (von Hippel-Lindau [VHL]) </li></ul><ul><li>i) autosomal dominant disease </li></ul><ul><li>ii) predispose to a variety of CA – hemangioblastoma of cerebellum and retina </li></ul>
  40. 42. <ul><li>iii) genetic abnormality chromosome 3, </li></ul><ul><li> which houses the VHL gene </li></ul><ul><li> (VHL gene acts as a tumor suppressor gene) – clear cell CA </li></ul><ul><li>Clinical </li></ul><ul><li>a) palpable mass, hematuria (most reliable), costovertebral pain </li></ul><ul><li>b) great mimicker (paraneoplastic syndromes) </li></ul><ul><li>i) polycythemia </li></ul><ul><li>ii) hypercalcemia </li></ul><ul><li>iii) hypertension </li></ul><ul><li>iv) Cushing syndrome </li></ul><ul><li>v) eosinophilis </li></ul>
  41. 43. <ul><li>common characteristic is to metastasize prior </li></ul><ul><li>to giving rise to symptoms </li></ul><ul><li>a) common site are the lungs (~ 50%) </li></ul><ul><li> and bones (~ 30%) </li></ul><ul><li>b) renal vein involvement (increases morbidity and mortality) </li></ul><ul><li>Renal cell CA are difficult to diagnose! </li></ul><ul><li>a) Present with hematuria in ~50% of cases </li></ul>
  42. 44. <ul><li>2.- Papillary renal cell Ca </li></ul><ul><li>10-15% of all renal CA </li></ul><ul><li>papillary growth pattern </li></ul><ul><li>Multifocal and bilateral </li></ul><ul><li>Both sporadic and familial forms </li></ul><ul><li>No genetic abnormalities in chromosome 3 </li></ul><ul><li>a) Protooncogene on chromosome 7 </li></ul>
  43. 45. <ul><li>3- Chromophobe Renal Carcinoma </li></ul><ul><li>Least common (~5% of all renal cell CA) </li></ul><ul><li>Cortical collecting ducts or their collated cells </li></ul><ul><li>Stain more darkly than clear cell CA </li></ul><ul><li>Lack of a lot of chromosomes (1,2,6,10,17,&21) </li></ul><ul><li>Have good prognosis vs clear cell and papillary </li></ul><ul><li>4.- Collecting duct (Bellini duct) carcinoma </li></ul><ul><li>~ 1% of renal epithelial cell carcinoma </li></ul><ul><li>arise from collecting duct in medulla </li></ul>
  44. 46. <ul><li>4. Wilms tumor (nephroblastoma) </li></ul><ul><li>Occurs infrequently in adults </li></ul><ul><li>1/3 most common organ cancer in children </li></ul><ul><li><15 years, (major cause in children) </li></ul><ul><li>a) most common 1  renal tumor of childhood </li></ul><ul><li>b) usually diagnosed between ages 2-5 yrs. </li></ul><ul><li>Sporadic or familial in nature </li></ul><ul><li>a) autosomal dominant </li></ul><ul><li>b) 3 groups at risk for developing </li></ul><ul><li>i) WAGR syngrome </li></ul><ul><li>ii) Denys-Drash syndrome (WT 1 gene) </li></ul><ul><li>iii) Beckwith-Wiedemann syndrome (WT 2 gene) </li></ul>
  45. 47. <ul><li>Clinical </li></ul><ul><li>Good outcome with early diagnosis. Tumor has </li></ul><ul><li>tendency to easily metastasize </li></ul><ul><li>major complaint is associated with large size of </li></ul><ul><li>the tumor </li></ul><ul><li>a) readily palpable mass </li></ul><ul><li>less common complaints include </li></ul><ul><ul><li>a) fever </li></ul></ul><ul><ul><li>b) abdominal pain </li></ul></ul><ul><ul><li>c) hematuria </li></ul></ul><ul><ul><li>d) intestinal obstruction (uncommon) </li></ul></ul>
  46. 48. <ul><li>Urothelial CA of renal pelvis </li></ul><ul><li>~ 5-10% of 1  renal tumors originate from </li></ul><ul><li>urothelium of renal pelvis </li></ul><ul><li>a) benign renal papillomas </li></ul><ul><li>b) invasive urothelial (transition cell) CA </li></ul><ul><li>become apparent quickly (hematuria) </li></ul><ul><ul><li>a) usually never palpable (small) </li></ul></ul><ul><li>In 50% there may be associated bladder </li></ul><ul><li>urothelial tumor </li></ul><ul><li>a) increased incidence in patients with </li></ul><ul><li>analgesic nephropathy </li></ul><ul><li>b) infiltration of pelvis and calyces is </li></ul><ul><li>common </li></ul><ul><li>i) prognosis not good </li></ul>
  47. 49. <ul><li>Urinary bladder and collecting system tumors (Renal pelvis to urethra) </li></ul><ul><li>Tumors in collecting system above bladder are </li></ul><ul><li>uncommon </li></ul><ul><li>Bladder Cancer more frequent cause of death </li></ul><ul><li>than are kidney tumors </li></ul><ul><li>a) Bladder tumors </li></ul><ul><li>i) small benign papillomas (rare) </li></ul><ul><li>ii) large invasive CA </li></ul><ul><li>iii) most recur after removal and kill by infiltrative obstruction of ureters rather than by metastasizing </li></ul><ul><li>iv) Shallow lesion have good prognosis </li></ul>
  48. 50. v) deep invasive Cancer, survival (5yr) is <20% with overall 5 yr survival at 50-60% b) Cancer of ureters is very rare i) 5 yr survival <10%

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