Tumors of the Kidney
Both benign and malignant tumors occur in the kidney.  With the exception of oncocytoma, the benign tumors rarely cause clinical problems. Malignant tumors, on the other hand,
are of great importance clinically and deserve considerable emphasis. By far the most common of these malignant tumors is renal cell carcinoma, followed by Wilms tumor, which is found
in children and is described in
Figure 20-57Nephrolithiasis. A large stone impacted in the renal pelvis. (Courtesy of Dr. E. Mosher, Brigham and Women's Hospital, Boston, MA.)
Figure 20-58Cytogenetics (blue) and genetics (red) of clear cell versus papillary renal cell carcinoma. (Courtesy of Dr. Keith Ligon, Brigham and Women's Hospital, Boston, MA.)
Figure 20-59Renal cell carcinoma. Typical cross-section of yellowish, spherical neoplasm in one pole of the kidney. Note the tumor in the dilated thrombosed renal vein.
Figure 20-60Renal cell carcinoma. A, Clear cell type, B, Papillary type. Note the papillae and foamy macrophages in the stalk. C, Chromophobe type. (Courtesy of Dr. A. Renshaw,
Brigham and Women's Hospital, Boston, MA.)
Figure 20-61Urothelial carcinoma of the renal pelvis. The pelvis has been opened to expose the nodular irregular neoplasm, just proximal to the ureter.
1. Dinesen I: Seven Gothic Tales. New York, Modern Library, 1939.
2. Kanwar YS, Venkatachalam MA: Morphology of the glomerulus and juxtaglomerular apparatus. In Handbook of Physiology, Section of Renal Physiology, 2nd ed. Washington, DC,
American Physiological Society, 1990.
3. Miner JH: Renal basement membrane components. Kidney Int 56:2016, 1999.
4. Timpl R, Brown JC: Supramolecular assembly of basement membranes. Bioassay 18:123, 1997.
5. Sundaramoorthy M, Meiyappan M, Todd P, Hudson BG: Crystal structure of NC1 domains. J Biol Chem 277:31142, 2002.
6. Tryggvason K, Wartiovaara J: Molecular basis of glomerular permselectivity. Curr Opin Nephrol Hypertens 10:543, 2001.
7. US Renal Data System, USRDS 2002 Annual Data Report: Atlas of End Stage Renal Disease in the United States. Bethesda, MD, National Institutes of Health, National Institute of
Diabetes and Digestive and Kidney Diseases, 2002.
8. National Center for Health Statistics: National Vital Statistics Report, vol 50, no 15, 2002.
9. Gardner KD Jr, Bernstein J: The Cystic Kidney. Dordrecht, Kluwer Academic Publishers, 1990.
10. Grantham JJ: The pathogenesis, etiology and treatment of autosomal dominant polycystic kidney disease. Am J Kidney Dis 28:788, 1996.
11. Hateboer N, et al: Comparison of phenotypes of polycystic kidney disease types 1 and 2. Lancet 353:103, 1999.
12. Rossetti S, et al: The position of the polycystic kidney disease 1 (PKD1) gene mutation correlates with the severity of renal disease. J Am Soc Nephrol 13:1230, 2002.
13. The International Polycystic Kidney Disease Consortium: Polycystic kidney disease: the complete structure of the PKD1 gene and its protein. Cell 81:289, 1995.
14. Wilson PD: Polycystic kidney disease. N Engl J Med 350:151, 2004.
15. Mochizuki T, et al: PKD2, a gene for polycystic kidney disease that encodes an integral membrane protein. Science 272:1339, 1996.
16. Koulen P, et al: Polycystin-2 is an intracellular calcium release channel. Nat Cell Biol 4:191, 2002.
17. Hanaoka K, et al: Co-assembly of polycystin-1 and -2 produces unique cation-permeable currents. Nature 408:990, 2000.
18. Lu W, et al: Perinatal lethality with kidney and pancreas defects in mice with a targeted PKD-1 mutation. Nat Gen 17:179, 1997.
19. Qian F, et al: The molecular basis of focal cyst formation in human autosomal dominant polycystic kidney disease type I. Cell 87:979, 1996.
20. Watson MC: Complications of APKD. Kidney Int 51:353, 1997.
21. Griffin MD, et al: Vascular expression of polycystin. J Am Soc Nephrol 8:616, 1997.
22. Ward CJ, et al: The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein. Nat Genet 30:259, 2002.
23. Hildebrandt F, Omram H: New insights: nephronophthisis-medullary cystic kidney disease. Pediatr Nephrol 16:168–176, 2001.
24. Drew AF, et al: Crescentic glomerulonephritis is diminished in fibrinogen-deficient mice. Am J Physiol Renal Physiol 281:F1157, 2001.
25. Nielsen EG, Couser WG: Immunologic Renal Diseases, 2nd ed. New York, Lippincott-Raven, 2001.
26. Wilson CB: Renal response to immunological injury. In Brenner BM, Rector F (eds): The Kidney, 5th ed. Philadelphia, WB Saunders, 1996, pp 1253–1391.
27. Hudson BG, Tryggvason K, Sundaramoorthy M, Neilson EG: Alport's syndrome, Goodpasture's syndrome, and type IV collagen. N Engl J Med 348:2543, 2003.
28. Farquhar M, et al: The Heymann nephritis antigenic complex: megalin (gp330) and RAP. J Am Soc Nephrol 6:35, 1996.
29. Ravetch JV, Lanier LL: Immune inhibitory receptors. Science 290:84, 2000.
30. Couser WG: Sensitized cells come of age: a new era in renal immunology with important therapeutic implications. J Am Soc Nephrol 10:664, 1999.
31. Cunard R, Kelly CJ: T cells and minimal change disease. J Am Soc Nephrol 13:1409, 2002.
32. Cunningham MA, et al: Prominence of cell-mediated immunity effectors in "pauci-immune" glomerulonephritis. J Am Soc Nephrol 10:499, 1999.
33. Rennke HG, et al: Cell-mediated immune injury in the kidney: acute nephritis induced in the rat by azobenzenearsonate. Kidney Int 45:1044, 1994.
34. Kalluri R, et al: Susceptibility to anti-glomerular basement membrane disease and Goodpasture syndrome is linked to MHC class II genes and emergence of T cell-mediated immunity
in mice. J Clin Invest 100:2263, 1997.
35. Tipping PG, et al: Crescentic glomerulonephritis in CD4- and CD8-deficient mice: requirement for CD4 but not CD8 cells. Am J Pathol 152:1541, 1998.
36. Johnson RJ: Cytokines, growth factors and renal injury. Kidney Int 52:S2, 1997.
37. Border WA, Noble NA: TGF-b in kidney fibrosis: a target for gene therapy. Kidney Int 51:1389, 1997.
38. Remuzzi G, Ruggenenti P, Benigni A: Understanding the nature of renal disease progression. Kidney Int 51:2, 1997.
39. Schena FP, et al: Progression of renal damage in human glomerulonephritis. Kidney Int 52:1439, 1997.
40. Fogo AB: Progression and potential regression of glomerulosclerosis. Kidney Int 59:804, 2001.
41. Rennke HG, et al: The progression of renal disease: structural and functional correlations. In Tisher CC, Brenner B (eds): Renal Pathology, 2nd ed. Philadelphia, JB Lippincott, 1994,
42. Brenner BM: Remission of renal disease: recounting the challenge, acquiring the goal. J Clin Invest 110:1753, 2003.
43. Mundel P, Shankland SJ: Podocyte biology and response to injury. J Am Soc Nephrol 13:3005, 2002.
44. Bohle A, et al: Pathogenesis of chronic renal failure in the primary glomerulopathies, renal vasculopathies, and chronic interstitial nephritides. Kidney Int Suppl 54:S2, 1996.
45. Abbate M, et al: Proteinuria as a mediator of tubulointerstitial injury. Kidney Blood Press Res 22:37, 1999.
46. Eddy AA: Molecular basis of renal fibrosis. Pediatr Nephrol 15:290, 2000.
47. Couser WG, et al: Postinfectious glomerulonephritis. In Neilson EG, Couser WG (eds): Immunologic Renal Diseases, 2nd ed. Philadelphia, Lippincott Williams & Wilkins, 2001, pp
48. Kluth DC, et al: Anti-glomerular basement membrane disease. J Am Soc Nephrol 10:2446, 1999.
49. Phelps RG, et al: The HLA complex in Goodpasture's disease: a model for analyzing susceptibility to autoimmunity. Kidney Int 56:1638, 1999.
50. Savige J, et al: Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features. Kidney Int 57:846, 2000.
51. Jennette JC, et al: Small-vessel vasculitis. N Engl J Med 337:1512, 1997.
52. Xiao H, et al: Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest 110:955, 2002.
53. Haas M, et al: Changing etiologies of unexplained adult nephrotic syndrome. Am J Kidney Dis 30:621, 1997.
54. Wasserstein AG: Membranous glomerulonephritis. J Am Soc Nephrol 8:664, 1997.
55. Burstein DM, et al: Membranous glomerulonephritis and malignancy. Am J Kidney Dis 22:5, 1993.
56. Schnaper HW: Primary nephrotic syndrome of childhood. Curr Opin Pediatr 8:141, 1996.
57. Tryggvason K: Unraveling the mechanisms of glomerular ultrafiltration: nephrin, a key component of the slit diaphragm. J Am Soc Nephrol 10:2440, 1999.
58. D'Agati V: The many masks of focal segmental glomerulosclerosis. Kidney Int 46:1223, 1994.
59. Laurinavicius A, Rennke HG: Collapsing glomerulopathy: a new pattern of renal injury. Semin Diagn Pathol 19:106, 2002.
60. Sharma M, et al: "The FSGS factor": enrichment and in vivo effect of activity from focal segmental glomerulosclerosis plasma. J Am Soc Nephrol 10:552, 1999.
61. Antignac C: Genetic models: clues for understanding the pathogenesis of idopathic nephrotic syndrome. J Clin Invest 109:447, 2002.
62. Pollak MR: Inherited podocytopathies: FSGS and nephrotic syndrome from a genetic viewpoint. J Am Soc Nephrol 13:3016, 2002.
63. Shaw AS, Miner JH: CD2-associated protein and the kidney. Curr Opin Nephrol Hypertens 10:19, 2001.
64. D'Agati V, Appel GB: HIV infection and the kidney. J Am Soc Nephrol 8:139, 1997.
65. Ross MJ, et al: Recent progress in HIV-associated nephropathy. J Am Soc Nephrol 13:2997, 2002.
66. Marras D, et al: Replication and compartmentalization of HIV-1 in kidney epithelium of patients with HIV-associated nephropathy. Nat Med 8:522, 2002.
67. Rennke HG: Secondary MPGN. Kidney Int 47:643, 1995.
68. Donadio JV, Grande JP: IgA nephropathy. N Engl J Med 347:738, 2002.
69. Kashtan CE: Alport syndrome: an inherited disorder of renal, ocular, and cochlear basement membranes. Medicine (Baltimore) 78:338, 1999.
70. Kashtan CE: Alport syndromes: phenotypic heterogeneity of progressive hereditary nephritis. Pediatr Nephrol 14:502, 2000.
71. Badenas C, et al: Mutations in the COLA4A4 and COLA4A3 genes cause familial benign hematuria. J Am Soc Nephrol 13:1248, 2002.
72. Ibrahim HN, Hostetter TH: Diabetic nephropathy. J Am Soc Nephrol 8:487, 1997.
73. Dalla Vestra M, et al: Structural involvement in type 1 and type 2 diabetic nephropathy. Diabetes Metab 26 (Suppl 4):8, 2000.
74. Sharma K, Ziyadeh FN: Biochemical events and cytokine interactions linking glucose metabolism to the development of diabetic nephropathy. Semin Nephrol 17:80, 1997.
75. Sheetz MJ, King GL: Molecular understanding of hyperglycemia's adverse effects for diabetic complications. JAMA 288:2579, 2002.
76. Wolf G, Ziyadeh FN: Molecular mechanisms of diabetic renal hypertrophy. Kidney Int 56:393, 1999.
77. Drummond K, Mauer M: The early natural history of nephropathy in type 1 diabetes: II. Early renal structural changes in type 1 diabetes. Diabetes 51:1580, 2002.
78. Fioretto P, et al: Reversal of lesions of diabetic nephropathy after pancreas transplantation. N Engl J Med 339:69, 1998.
79. Rosenstock JL, et al: Fibrillary and immunotactoid glomerulonephritis: distinct entities with different clinical and pathologic features. Kidney Int 63:1450, 2003.
80. Lieberthal WL: Biology of acute renal failure. Kidney Int 52:1102, 1997.
81. Lameire N, Vanholder R: Pathophysiologic features and prevention of human and experimental acute tubular necrosis. J Am Soc Nephrol 12 (Suppl 17):S20, 2001.
82. Edelstein CL, et al: The nature of renal cell injury. Kidney Int 51:341, 1997.
83. Rabb H, et al: Leukocytes, cell adhesion molecules and ischemic renal failure. Kidney Int 51:1463, 1997.
84. Humes DH, et al: Acute renal failure: growth factors, cell therapy and gene therapy. Proc Am Assoc Physicians 109:547, 1997.
85. Oliver J, et al: The pathogenesis of acute renal failure associated with traumatic and toxic injury, renal ischemia, nephrotoxic damage, and the ischemic episode. J Clin Invest 30:1307,
86. Ronald A: The etiology of urinary tract infection: traditional and emerging pathogens. Am J Med 113 (Suppl 1A):14S, 2002.
87. Langermann S, et al: Prevention of mucosal Escherichia coli infection by FimH-adhesin-based systemic vaccination. Science 276:607, 1997.
88. Hirsch HH: Polyomavirus BK nephropathy: a (re-)emerging complication in renal transplantation. Am J Transplant 2:25, 2002.
89. Michel DM, Kelly CJ: Acute interstitial nephritis. J Am Soc Nephrol 9:506, 1998.
90. De Broe ME, Elseveirs MM: Analgesic nephropathy. N Engl J Med 338:446, 1998.
91. Gambaro G, Perazella MA: Adverse renal effects of anti-inflammatory agents: evaluation of selective and non-selective cyclooxygenase inhibitors. J Intern Med 253:643, 2003.
92. Kurokawa K, et al (eds): Hypertension: causes and consequences of renal injury. Kidney Int 49 (Suppl 55):S1, 1997.
93. Preston RA, et al: Renal parenchymal hypertension: present concepts. Arch Intern Med 156:602, 1996.
94. Meyrier A, et al: Ischemic renal diseases: new insights into old entities. Kidney Int 54:2, 1998.
95. Kitiyakara C, Guzman NJ: Malignant hypertension and hypertensive emergencies. J Am Soc Nephrol 9:128, 1998.
96. Safian RD, Textor SC: Renal-artery stenosis. N Engl J Med 344:431, 2001.
97. Goldblatt H, et al: Studies on experimental hypertension: I. Production of persistent elevation of systolic blood pressure by means of renal ischemia. J Exp Med 59:347, 1934.
98. Elliott MA, Nichols WL: Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Mayo Clin Proc 76:1154, 2001.
99. Moake JL: Thrombotic microangiopathies. N Engl J Med 347:589, 2002.
100. Chung DW, Fujikawa K: Processing of von Willebrand factor by ADAMTS-13. Biochemistry 41:11065, 2002.
101. Grabowski EF: The hemolytic-uremic syndrome—toxin, thrombin and thrombosis. New Engl J Med 346:58, 2002.
102. Gagnuandou MF, et al: Long-term (15–25 years) prognosis of hemolytic-uremic syndrome. J Am Soc Nephrol 4:275, 1993.
103. Textor SC, Wilcox CS: Ischemic nephropathy/azotemic renovascular disease. Semin Nephrol 20:489, 2000.
104. Klahr S: Obstructive nephropathy. Kidney Int 54:286, 1998.
105. Scheinman SJ: Nephrolithiasis. Semin Nephrol 19:381, 1999.
106. Coe FL, Parks JH: New insights into the pathophysiology and treatment of nephrolithiasis: new research venues. J Bone Miner Res 12:522, 1997.
107. Pak CY: Kidney stones. Lancet 351:1797, 1998.
108. Eble JN (ed): Tumors of the kidney. Semin Diagn Pathol 15:1–81, 1998.
109. Reuter VE, Presti JC, Jr.: Contemporary approach to the classification of renal epithelial tumors. Semin Oncol 27:124, 2000.
110. McLaughlin JK, Lipworth L: Epidemiologic aspects of renal cell cancer. Semin Oncol 27:115, 2000.
111. Bodmer D, et al: Understanding familial and non-familial renal cell cancer. Hum Mol Genet 11:2489, 2002.
112. Karumanchi SA, Merchan J, Sukhatme VP: Renal cancer: molecular mechanisms and newer therapeutic options. Curr Opin Nephrol Hypertens 11:37, 2002.
113. Pavlovich CP, Schmidt LS, Phillips JL: The genetic basis of renal cell carcinoma. Urol Clin North Am 30:437, 2003.
114. Weterman MA, et al: Impairment of MAD2B-PRCC interaction in mitotic checkpoint defective t(X;1)-positive renal cell carcinomas. Proc Natl Acad Sci U S A 98:13808, 2001.
Date: 2016-04-22; view: 228