Skip to main content
SpringerLink
Log in

Management of Patients with Pathologic Variants of Renal Cell Carcinoma: Papillary, Collecting Duct, Medullary and Chromophobe Carcinoma, and Sarcomatoid Differentiation

  • Chapter
Clinical Management of Renal Tumors

  • 764 Accesses

Abstract

Non-clear-cell renal cell carcinoma is an uncommon disease with distinct subtypes. These subtypes have different pathologic characteristics and biologic behaviors, including occasional responses to chemotherapy and possibly the new targeted therapies. This chapter focuses on the evaluation and management of papillary, collecting duct, medullary, and chromophobe carcinoma as well as sarcomatoid variant. Current treatment evidence relies on small cohort studies. As such, it is important to consider well-designed trials, with an emphasis on subtype of non-clear-cell carcinoma, as first-line therapy.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Chow WH, Devesa SS, Warren JL, Fraumeni JF Jr. Rising incidence of renal cell cancer in the United States. JAMA 1999;281:1628–1631.

    Article  PubMed  CAS  Google Scholar 

  2. Reuter VE, Presti JC. Contemporary approach to the classification of renal epithelial tumors. Semin Oncol 2000;27:124–137.

    PubMed  CAS  Google Scholar 

  3. Storkel S, Eble JN, Adlakha K, et al. Classification of renal cell carcinoma: Workgroup No. 1 Union Internationale Contre le Cancer (UICC) and the American Joint Committee on Cancer (AHCC). Cancer 1997;80:987–989.

    Article  PubMed  CAS  Google Scholar 

  4. Kovacs G, Akhtar M, Beckwith BJ, et al. The Heidelberg classification of renal cell tumours. J Pathol 1997;183:131–133.

    Article  PubMed  CAS  Google Scholar 

  5. Beck SD, Patel MI, Snyder ME, et al. Effect of papillary and chromophobe cell type on disease-free survival after nephrectomy for renal cell carcinoma. Ann Surg Oncol 2004;11:71–77.

    Article  PubMed  Google Scholar 

  6. Atkins MB, Dutcher J, Weiss G, et al. Kidney cancer: the Cytokine Working Group experience (1986–2001): part I. IL-2 based clinical trials. Med Oncol 2001;18(3):197–207.

    Article  PubMed  CAS  Google Scholar 

  7. Levin HS, Myles JL. The pathology of renal neoplasms. In: Bukowski RM, Novick AC, eds. Renal Cell Carcinoma. Totowa, NJ: Humana Press, 2000:15–38.

    Chapter  Google Scholar 

  8. Yang XJ, Sugimura J, Tretiakova MS, et al. Gene expression profiling of renal medullary carcinoma. Cancer 2004;100:976–985.

    Article  PubMed  Google Scholar 

  9. Takahashi M, Teh BT. Gene expression profiling of renal cell carcinoma and its clinical implications. In: Ladanyi M, Gerald WL, eds. Expression Profiling of Human Tumors: Diagnostic and Research Applications. Totowa, NJ: Humana Press, 2003:235–256.

    Chapter  Google Scholar 

  10. Sanders ME, Mick R, Tomaszewski JE, et al. Unique patterns of allelic imbalance distinguish type I from type II sporadic papillary renal cell carcinoma. Am J Pathol 2002;161:997–1005.

    PubMed  Google Scholar 

  11. Young AN, Amin MB, Moreno CS, et al. Expression profiling of renal epithelial neoplasms: a method for tumor classification and discovery of diagnostic molecular markers. Am J Pathol 2001;158:1639–1651.

    PubMed  CAS  Google Scholar 

  12. Linehan WM, Walther MM, Zbar B. Genetic basis of cancer of the kidney. J Urol 2003; 170:2163–2172.

    Article  PubMed  CAS  Google Scholar 

  13. Schmidt L, Duh FM, Chen F, et al. Germline and somatic mutations in the tyrosine kinase domain of the MET proto-oncogene in papillary renal carcinomas. Nat Genet 1997;16:68–73.

    Article  PubMed  CAS  Google Scholar 

  14. Pavlovich CP, Walther MM, Eyler RA, et al. Renal tumors in the Birt-Hogg-Dube syndrome. Am J Surg Pathol 2002;26(12):1542–1552.

    Article  PubMed  Google Scholar 

  15. Cheville JC, Lohse CM, Zincke H, et al. Comparisons of outcome and prognostic features among histologic subtypes of renal cell carcinoma. Am J Surg Pathol 2003;27(5):612–624.

    Article  PubMed  Google Scholar 

  16. Davis CJ, Mostofi FK, Sesterhenn IA, Renal medullary Carcinoma. The seventh sickle cell nephropathy. Am J Surg Pathol 1995;19:1–11.

    Article  PubMed  Google Scholar 

  17. Swartz MA, Karth J, Schneider DT, et al. Renal medullary carcinoma: clinical, pathologic, immuno-histochemical, and genetic analysis with pathogenic implications. Urology 2002;60:1083–1089.

    Article  PubMed  Google Scholar 

  18. Guinan P, Sobin LH, Algaba F, et al. TNM staging of renal cell carcinoma: workgroup No. 3. Union International Contre le Cancer (UICC) and the American Joint Committee on Cancer (AJCC). Cancer 1997;80:992–993.

    Article  PubMed  CAS  Google Scholar 

  19. Majean A, Roupret M, Larousserie F, et al. Is there a place for radical nephrectomy in the presence of metastatic collecting duct carcinoma? J Urol 2003;169(4):1287–1290.

    Article  Google Scholar 

  20. Crotty TB, Farrow GM, Lieber MM. Chromophobe cell carcinoma: clinicopathological features of 50 cases. J Urol 1995;154(3):964–967.

    Article  PubMed  CAS  Google Scholar 

  21. Sanchez-Ortiz RF, Rosser CJ, Madsen TL, et al. Young age is an independent prognostic factor for survival of sporadic renal cell carcinoma. J Urol 2004;171(6 pt 1):2160–2165.

    Article  PubMed  Google Scholar 

  22. Motzer RJ, Mazumdar M, Bacik J, Berg W, Amsterdam A, Ferrara J. Survival and prognostic stratification of 670 patients with advanced renal cell carcinoma. J Clin Oncol 1999;17(8):2530–2540.

    PubMed  CAS  Google Scholar 

  23. Mekhail TM, Abou-Jawde RM, BouMerhi G, et al. Validation and extension of the Memorial Sloan-Kettering prognostic factors model for survival in patients with previously untreated metastatic renal cell carcinoma. J Clin Oncol 2005;23(4):832–841.

    Article  PubMed  Google Scholar 

  24. Patard JJ, Leray E, Rioux-Leclerq N, et al. Prognostic value of histologic subtypes in renal cell carcinoma: a multicenter experience. J Clin Oncol 2005;23(12):2763–2771.

    Article  PubMed  Google Scholar 

  25. Elson PJ, Manola JB, Mazumdar M, et al. Prognostic factors for survival in patients with metastatic renal cell carcinoma: a study from the Kidney Cancer Association’s International Kidney Cancer Working Group. Proc Am Soc Clin Oncol 2005;24:abstr 4533.

    Google Scholar 

  26. Gold PJ, Fefer A, Thompson JA. Paraneoplastic manifestations of renal cell carcinoma. Semin Urol Oncol 1996;14:216–222.

    PubMed  CAS  Google Scholar 

  27. Weiss LM, Gelb AB, Medeiros LJ. Adult renal epithelial neoplasms. Am J Clin Pathol 1995; 103:624–635.

    PubMed  CAS  Google Scholar 

  28. Frank I, Blute ML, Cheville JC, et al. A multifactorial postoperative surveillance model for patients with surgically treated clear cell renal cell carcinoma. Urology 2003;170(6 pt 1):2225–2232.

    Google Scholar 

  29. Motzer RJ, Bacik J, Mazumdar M. Prognostic factors for survival of patients with stage IV renal cell carcinoma: Memorial Sloan-Kettering Cancer Center experience. Clin Cancer Res 2004;10(18 pt 2):6302S–6303S.

    Article  PubMed  CAS  Google Scholar 

  30. Ljunberg B, Alamdari FL, Stenling R, et al. Prognostic significance of the Heidelberg classification of renal cell carcinoma. Eur Urol 1999;36(6):565–569.

    Article  Google Scholar 

  31. Amin MB, Tamboli P, Javidan J, et al. Prognostic impact of histologic subtyping of adult renal epithelial neoplasms: an experience of 405 cases. Am J Surg Pathol 2002;26(3):281–291.

    Article  PubMed  Google Scholar 

  32. Noguera-Irizarry WG, Hibshoosh H, Papadopoulos KP. Renal medullary carcinoma: case report and review of the literature. Am J Clin Oncol 2003;26(5):489–492.

    Article  PubMed  Google Scholar 

  33. Selby DM, Simon C, Foley JP, Thompson IM, Baddour RT. Renal medullary carcinoma: can early diagnosis lead to long-term survival? J Urol 2000;163(4):1238.

    Article  PubMed  CAS  Google Scholar 

  34. Upton MP, Parker RA, Youmans, et al. Histologic predictors of renal cell carcinoma (RCC) response to interleukin 2 based therapy. Proc Am Soc Clin Oncol 2003;22:851.

    Google Scholar 

  35. Motzer RJ, Bacik J, Mariani T, et al. Treatment outcome and survival associated with metastatic renal cell carcinoma of non-clear-cell histology. J Clin Oncol 2002;20:2376–2381.

    Article  PubMed  Google Scholar 

  36. Saranchuk JW, Touijer AK, Hakiman, et al. Partial Nephrectomy for patients with solitary kidney: the memorial Sloan-Kettering experience. BJU Int 2004;94(9):1323–1328.

    Article  PubMed  Google Scholar 

  37. Allaf ME, Bhayani SB, Rogers C, Varkarakis I. Laparoscopic partial nephrectomy: evaluation of long term oncological outcome. J Urol 2004;172(3):871–873.

    Article  PubMed  Google Scholar 

  38. Wronski M, Arbit E, Russo P, et al. Surgical resection of brain metastases from renal cell carcinoma in 50 patients. Urology 1996;47(92):187–193.

    Article  PubMed  CAS  Google Scholar 

  39. Flanigan RC. Debulking nephrectomy in metastatic renal cell cancer. Clin Cancer Res 2004;10(18 pt 2):6335S–6341S.

    Article  PubMed  Google Scholar 

  40. Wood CG. The role of cytoreductive nephrectomy in the management of metastatic renal cell carcinoma. Urol Clin North Am 2003;30(3):581–588.

    Article  PubMed  Google Scholar 

  41. Mickish GHJ, Garin A, van Poppel H, et al. Radical nephrectomy plus interferon-alfa-based immunotherapy compared with interferon alfa alone in metastatic renal cell carcinoma: a randomized trial. Lancet 2001;358(9286):966–970.

    Article  Google Scholar 

  42. Flanigan RC, Salmon SE, Blumenstein BA, et al. Nephrectomy followed by interferon alfa compared with interferon alfa alone for metastatic renal cell cancer. N Engl J Med 2001;23(345):1655–1659.

    Article  Google Scholar 

  43. Sella A, Logothetis CJ, Ro JY, Swanson DA, Samuels ML. Sarcomatoid renal cell carcinoma. A treatable entity. Cancer 1987;60(6):1313–1318.

    Article  PubMed  CAS  Google Scholar 

  44. Escudier B, Droz JP, Rolland F, Terrier-Lacombe MJ, Gravis G, Beuzeboc P. Doxorubicin and Ifosfamide in patients with metastatic sarcomatoid renal cell carcinoma: a phase II study of the Genitourinary Group of the French Federation of Cancer Centers. J Urol 2002;168(3):959–961.

    Article  PubMed  CAS  Google Scholar 

  45. Nanus DM, Garino A, Milowski MI, Larkin M, Dutcher JP. Active chemotherapy for sarcomatoid and rapidly progressing renal cell carcinoma. Cancer 2004;101(7):1545–1551.

    Article  PubMed  CAS  Google Scholar 

  46. Hoshi S, Satoh M, Ohyama C, et al. Active chemotherapy for bone metastasis in sarcomatoid renal cell carcinoma. Int J Clin Oncol 2003;8(2):113–117.

    Article  PubMed  Google Scholar 

  47. Bangalore N, Bhargawa P, Hawkins, et al. Sustained response of sarcomatoid renal-cell carcinoma to MAID chemotherapy: case report and review of the literature. Ann Oncol 2001;12(2):271–274.

    Article  PubMed  CAS  Google Scholar 

  48. Pirich LM, Chou P, Walterhouse DO. Prolonged survival of a patient with sickle cell trait and metastatic renal medullary carcinoma. Pediatr Hematol Oncol 1999;21(1):67–69.

    Article  CAS  Google Scholar 

  49. Warren KE, Gidvani-Diaz V, Duval-Arnould B. Renal medullary carcinoma in an adolescent with sickle cell trait. Pediatrics 1999;103:22–25.

    Article  Google Scholar 

  50. Vargas-Gonzales R, Sotelo-Avila C, Coria AS. Renal medullary carcinoma in a six-year-old boy with sickle cell trait. Pathol Oncol Res 2003;9:193–195.

    Article  Google Scholar 

  51. Strousse JJ, Spevak M, Kyle Mack A, Arceci RJ. Significant responses to platinum based chemotherapy in renal medullary carcinoma. Pediatr Blood Cancer 2005;44:1–5.

    Article  Google Scholar 

  52. Dimopoulos MA, Logothetis CJ, Markowitz A, Sella A, Amato R, Ro J. Collecting duct carcinoma of the kidney. Br J Urol 1993;71(4):388–391.

    Article  PubMed  CAS  Google Scholar 

  53. Peyromaure M, Thiounn N, Scotte F, Vieillefond A, Debre B, Oudard S. Collecting duct carcinoma of the kidney: a clinicopathological study of 9 cases. Urology 2003;170(4 pt 1):1138–1140.

    Google Scholar 

  54. Oudard S, Banu E, Viellefond A, et al. Prospective multicenter phase II study of gemcitabine plus platinum salt for metastatic collecting duct carcinoma: Results of a GETUG (Groupe d’Etudes des Tumeurs Uro-Genitales) study. J Urol 2007;177(5):1698–1702.

    Article  PubMed  CAS  Google Scholar 

  55. Gollob JA, Upton MP, DeWolf WC, Atkins MB. Long-term remission in a patient with metastatic collecting duct carcinoma treated with Taxol/carboplatin and surgery. Urology 2001;58(6):1058.

    Article  PubMed  CAS  Google Scholar 

  56. Milowsky MI, Rosmarin A, Tickoo SK, et al. Active chemotherapy for collecting duct carcinoma of the kidney: a case report and review of the literature. Cancer 2002;94(1):111–116.

    Article  PubMed  Google Scholar 

  57. Stadler WM, Halabi S, Rini B, et al. A phase II study of gemcitabine and capecitabine in metastatic renal cancer: A report of Cancer and Leukemia Group B Protocol 90008. Cancer 2006; 107:1273–1279.

    Article  PubMed  CAS  Google Scholar 

  58. Posadas EM, Undevia S, Manchen E, et al. A phase II study of Ixabepilone (BMS-247550) in metastatic renal cell carcinoma. Cancer Biol Ther 2007;5:490–493.

    Google Scholar 

  59. Moch H, Sauter G, Buchholz N, Gasser TC, Bubendorf L, Waldman FM. Epidermal growth factor receptor expression is associated with rapid tumor cell proliferation in renal cell carcinoma. Hum Pathol 1997;28(11):1255–1259.

    Article  PubMed  CAS  Google Scholar 

  60. Dawson NA, Guo C, Zak R, Dorsey B, Hussain,. A phase II trial of gefitinib (Iressa, ZD1839) in stage IV and recurrent renal cell carcinoma. Clin Cancer Res 2004;10(23):7812–7819.

    Article  PubMed  CAS  Google Scholar 

  61. Motzer RJ, Amato R, Todd M, Hwu WJ. Phase II trial of antiepidermal growth factor receptor antibody C225 in patients with advanced renal cell carcinoma. Invest New Drugs 2003;21(1):99–101.

    Article  PubMed  CAS  Google Scholar 

  62. Rowinsky EK, Schwartz GH, Gollob JA, et al. Safety, pharmacokinetics, and activity of ABX-EGF, a fully human anti-epidermal growth factor receptor monoclonal antibody in patients with metastatic renal cell cancer. J Clin Oncol 2004;22(15):3003–3015.

    Article  PubMed  CAS  Google Scholar 

  63. Pan C, Hussey M, Lara P, et al. Phase II trial of the epidermal growth factor receptor (EGFR) inhibitor erlotinib (e) in patients with advanced papillary renal cell carcinoma—SWOG S0317. J Clin Oncol 2007; ASCO Annual Meeting Proceedings, 25(18S):15516.

    Google Scholar 

  64. Perera AD, Kleymenova EV, Walker CL. Requirement for the von Hippel-Lindau tumor suppressor gene for functional epidermal growth factor receptor blockade by monoclonal antibody C225 in renal cell carcinoma. Clin Can Res 2000;6:1518–1523.

    CAS  Google Scholar 

  65. Sumimoto M, Asano T, Asakuma J, Asano T. ZD1839 modulates paclitaxel response in renal cancer by blocking paclitaxel-induced activation of epidermal growth factor receptor-extracellular signal-regulated kinase pathway. Clin Cancer Res 2004;1092:794–801.

    Article  Google Scholar 

  66. Lin ZH, Han EM, Lee ES, Kim CW. A distinct expression pattern and point mutation of c-kit in papillary renal cell carcinoma. Mod Pathol 2004;17(6):611–616.

    Article  PubMed  CAS  Google Scholar 

  67. Petit A, Castillo M, Santos M, Mallofre C. KIT expression in chromophobe renal cell carcinoma: comparative immunohistochemical analysis of KIT expression in different renal cell neoplasms. Am J Surg Pathol 2004;28(5):676–678.

    PubMed  Google Scholar 

  68. Yamazaaki K, Sakamoto M, Ohta T, Kanai Y. Overexpression of KIT in chromophobe renal cell carcinoma. Oncogene 2003;22(6):847–852.

    Article  CAS  Google Scholar 

  69. Castillo M, Petit A, Mellado B, Palacin A. C-kit expression in, sarcomatoid renal cell carcinoma therapy with imatinib. J Urol 2004;171(6 pt 1):2176–2180.

    Article  PubMed  CAS  Google Scholar 

  70. Sihto H, Rikala MS, Tynninen O, et al. KIT and Platelet-derived growth factor receptor alpha tyrosine kinase gene mutations and KIT amplifications in human solid tumors. J Clin Oncol 2005;23:49–57.

    Article  PubMed  CAS  Google Scholar 

  71. Jacobsen J, Grankvist K, Rasmuson T, et al. Expression of vascular endothelial growth factor protein in human renal cell carcinoma. BJU Int 2004;93(3):297–302.

    Article  PubMed  CAS  Google Scholar 

  72. Yang JC, Haworth L, Sherry RM, et al. A randomized trial of bevacizumab, an anti-vascular endothelial growth factor antibody, for metastatic renal cancer. N Engl J Med 2003;349(5):427–434.

    Article  PubMed  CAS  Google Scholar 

  73. Stadler WM, Figlin RA, Ernstoff MS, et al. The Advanced Renal Cell Carcinoma Sorafenib (ARCCS) expanded access trial: Safety and efficacy in patients with non-clear cell renal cell carcinoma. ASCO Annual Meeting Proceedings. J Clin Oncol 2007;25(18S):5036.

    Google Scholar 

  74. Knox JJ, Figlin RA, Stadler WM, et al. The Advanced Renal Cell Carcinoma Sorafenib (ARCCS) expanded access trial in North America: Safety and efficacy. ASCO Annual Meeting Proceedings. J Clin Oncol 2007;25(18S):5011.

    Google Scholar 

  75. Gore ME, Porta C, Oudard S, et al. Sunitinib in metastatic renal cell carcinoma: Preliminary assessment in an expanded access trial with subpopulation analysis. ASCO Annual Meeting Proceedings. J Clin Oncol 2007;25(18S):5010.

    Google Scholar 

  76. Gore ME, Szczylik C, Porta C, et al. Sunitinib in metastatic renal cell carcinoma: Preliminary assessment in an expanded access trial with subpopulation analysis. Proc ECCO, 2007;10:45.

    Google Scholar 

  77. Aoki M, Blazek E, Vogt PK, et al. A role of the kinase mTOR in cellular transformation induced by oncoproteins PI3K and AKT. Proc Natl Acad Sci U S A 2001;98:136–141.

    Article  PubMed  CAS  Google Scholar 

  78. Schmelzle T, Hall MN. TOR, a central controller of cell growth. Cell 2000;103:253–262.

    Article  PubMed  CAS  Google Scholar 

  79. Castedo M, Ferri KF, Kroemer G. Mammalian target of rapamycin (mTOR). Pro-and Anti-apoptotic Cell Death Differ 2002;9:99–100.

    Article  CAS  Google Scholar 

  80. Atkins MB, Hidalgo M, Stadler WM, et al. Randomized phase II study of multiple dose levels of CCI-779, a novel mammalian target of rapamycin kinase inhibitor, in patients with advanced refractory renal cell carcinoma. J Clin Oncol 2004;22(5):909–918.

    Article  PubMed  CAS  Google Scholar 

  81. Hudes G, Hudes G, Carducci M, et al. Temsirolimus, interferon, or the combination of interferon plus temsirolimus for patients with advanced renal cell carcinoma and poor risk features. N Engl J Med 2007;356:2271–2281.

    Article  PubMed  CAS  Google Scholar 

  82. Dutcher J, Szczylik C, Tannir N, et al. Correlation of survival with tumor histology, age and prognostic-risk group for previously untreated patients with advanced renal cell carcinoma receiving temsirolimus or interferon alpha. ASCO Annual Meeting Proceedings. J Clin Oncol, 2007;25(18S):5033.

    Google Scholar 

  83. Kondagunta GV, Drucker B, Schwartz L, et al. Phase II trial of bortezomib for patients with advanced renal cell carcinoma. J Clin Oncol 2004;22:3720–3725.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Our Lady of Mercy Medical Center, Comprehensive Cancer Center, Bronx, NY

    Vladimir Hugec MD & Janice P. Dutcher MD

Authors
  1. Vladimir Hugec MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Janice P. Dutcher MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Taussig Cancer Center, Cleveland Clinic Foundation, Cleveland, OH

    Ronald M. Bukowski MD

  2. Glickman Urological and Kidney Institute, Cleveland Clinic Foundation, Cleveland, OH

    Andrew C. Novick MD

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Humana Press Inc., Totowa, NJ

About this chapter

Cite this chapter

Hugec, V., Dutcher, J.P. (2008). Management of Patients with Pathologic Variants of Renal Cell Carcinoma: Papillary, Collecting Duct, Medullary and Chromophobe Carcinoma, and Sarcomatoid Differentiation . In: Bukowski, R.M., Novick, A.C. (eds) Clinical Management of Renal Tumors. Humana Press. https://doi.org/10.1007/978-1-60327-149-3_32

Download citation

  • DOI: https://doi.org/10.1007/978-1-60327-149-3_32

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-251-3

  • Online ISBN: 978-1-60327-149-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation