The Kidney pp 109-123 | Cite as

Collecting Duct Carcinoma and Renal Medullary Carcinoma

  • Jamie Koo
  • Christopher P. Filson
  • Jiaoti Huang
  • Allan J. PantuckEmail author


Collecting duct carcinoma (CDC) and renal medullary carcinoma (RMC) are both rare and distinct subtypes of renal cell carcinoma (RCC) arising from the distal segment of the collecting ducts. Patients generally present with high-stage disease and median survival is significantly shorter than patients with more conventional RCC subtypes. RMC commonly affects younger, African American patients and almost all patients have a sickle cell hemoglobinopathy, most commonly sickle cell trait. Differential diagnoses include urothelial carcinoma, papillary RCC, and metastatic carcinoma. Patients with CDC and RMC are generally treated with surgical excision, followed by systemic chemotherapy, even though these tumors generally respond poorly to chemotherapy. However, due to the rarity of these tumors, studies of treatment outcome are limited to small case series and case reports, and more studies are needed to improve the outcome for these patients.


Collecting duct carcinoma Renal medullary carcinoma Renal cell carcinoma Immunohistochemistry Clinical management 


  1. 1.
    Davis CJ, Mostofi FK, Sesterhenn IA. Renal medullary carcinoma. The seventh sickle cell nephropathy. Am J Surg Pathol. 1995;19(1):1–11.PubMedCrossRefGoogle Scholar
  2. 2.
    Srigley JR, Moch H. Carcinoma of the collecting ducts of Bellini. In: Eble JN, Sauter G, Epstein JI, Sesterhenn IA, editors. World Health Organization classification of tumors pathology and genetics: tumors of the urinary system and male genital organs. Lyon: IARC; 2004. p. 33–4.Google Scholar
  3. 3.
    Abern MR, Tsivian M, Polascik TJ, Coogan CL. Characteristics and outcomes of tumors arising from the distal nephron. Urology. 2012;80(1):140–6.PubMedCrossRefGoogle Scholar
  4. 4.
    Tokuda N, Naito S, Matsuzaki O, Nagashima Y. Collecting duct (Bellini duct) renal cell carcinoma: a nationwide survey in Japan. J Urol. 2006;176(1):40–3.PubMedCrossRefGoogle Scholar
  5. 5.
    Simpson L, He X, Pins M, Huang X, Campbell S, Yang X, et al. Renal medullary carcinoma and abl gene amplification. J Urol. 2005;173(6):1883–8.PubMedCrossRefGoogle Scholar
  6. 6.
    Wright JL, Risk MC, Hotaling J, Lin DW. Effect of collecting duct histology on renal cell cancer outcome. J Urol. 2009;182(6):2595–600.PubMedCentralPubMedCrossRefGoogle Scholar
  7. 7.
    Srigley JR, Eble JN. Collecting duct carcinoma of kidney. Semin Diagn Pathol. 1998;15(1):54–67.PubMedGoogle Scholar
  8. 8.
    Gatalica Z, Lilleberg SL, Monzon FA, Koul MS, Bridge JA, Knezetic J, et al. Renal medullary carcinomas: histopathologic phenotype associated with diverse genotypes. Hum Pathol. 2011;42(12):1979–88.PubMedCrossRefGoogle Scholar
  9. 9.
    Watanabe IC, Billis A, Guimarães MS, Alvarenga M, de Matos AC, Cardinalli IA, et al. Renal medullary carcinoma: report of seven cases from Brazil. Mod Pathol. 2007;20(9):914–20.PubMedCrossRefGoogle Scholar
  10. 10.
    Yoon SK, Nam KJ, Rha S-H, Kim JK, Cho K-S, Kim B, et al. Collecting duct carcinoma of the kidney: CT and pathologic correlation. Eur J Radiol. 2006;57(3):453–60.PubMedCrossRefGoogle Scholar
  11. 11.
    Prasad SR, Humphrey PA, Catena JR, Narra VR, Srigley JR, Cortez AD, et al. Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation. Radiographics. 2006;26(6):1795–806.PubMedCrossRefGoogle Scholar
  12. 12.
    Blitman NM, Berkenblit RG, Rozenblit AM, Levin TL. Renal medullary carcinoma: CT and MRI features. Am J Roentgenol. 2005;185(1):268–72.CrossRefGoogle Scholar
  13. 13.
    Gupta R, Billis A, Shah RB, Moch H, Osunkoya AO, Jochum W, et al. Carcinoma of the collecting ducts of Bellini and renal medullary carcinoma. Am J Surg Pathol. 2012;36(9):1265–78.PubMedCrossRefGoogle Scholar
  14. 14.
    Tickoo AK, Reuter VE. Collecting duct carcinoma and renal medullary carcinoma. In: Amin MB, McKenney JK, Tickoo SK, editors. Diagnostic pathology: genitourinary. Manitoba: Amirsys; 2010. p. 64–75.Google Scholar
  15. 15.
    Srigley JR, Delahunt B, Eble JN, Egevad L, Epstein JI, Grignon D, et al. The International Society of Urological Pathology (ISUP) Vancouver classification of renal neoplasia. Am J Surg Pathol. 2013;37(10):1469–89.PubMedCrossRefGoogle Scholar
  16. 16.
    Kobayashi N, Matsuzaki O, Shirai S, Aoki I, Yao M, Nagashima Y. Collecting duct carcinoma of the kidney: an immunohistochemical evaluation of the use of antibodies for differential diagnosis. Hum Pathol. 2008;39(9):1350–9.PubMedCrossRefGoogle Scholar
  17. 17.
    Albadine R, Schultz L, Illei P, Ertoy D, Hicks J, Sharma R, et al. PAX8 (+)/p63 (−) Immunostaining pattern in renal collecting duct carcinoma (CDC). Am J Surg Pathol. 2010;34(7):965–9.PubMedCentralPubMedCrossRefGoogle Scholar
  18. 18.
    Srigley JR, Delahunt B. Uncommon and recently described renal carcinomas. Mod Pathol. 2009;22:S2–23.PubMedCrossRefGoogle Scholar
  19. 19.
    Elwood H, Chaux A, Schultz L, Illei PB, Baydar DE, Billis A, et al. Immunohistochemical analysis of SMARCB1/INI-1 expression in collecting duct carcinoma. Urology. 2011;78(2):474.e1–e5.CrossRefGoogle Scholar
  20. 20.
    Calderaro J, Moroch J, Pierron G, Pedeutour F, Grison C, Maillé P, et al. SMARCB1/INI1 inactivation in renal medullary carcinoma. Histopathology. 2012;61(3):428–35.PubMedCrossRefGoogle Scholar
  21. 21.
    Fuzesi L, Cober M, Mittermayer CH. Collecting duct carcinoma: cytogenetic characterization. Histopathology. 1992;21(2):155–60.PubMedCrossRefGoogle Scholar
  22. 22.
    Cavazzana AO, Prayer-Galetti T, Tirabosco R, Macciomei MC, Stella M, Lania L, et al. Bellini duct carcinoma. A clinical and in vitro study. Eur Urol. 1996;30(3):340–4.PubMedGoogle Scholar
  23. 23.
    Antonelli A, Portesi E, Cozzoli A, Zanotelli T, Tardanico R, Balzarini P, et al. The collecting duct carcinoma of the kidney: a cytogenetical study. Eur Urol. 2003;43(6):680–5.PubMedCrossRefGoogle Scholar
  24. 24.
    Gregori-Romero MA, Morell-Quadreny L, Llombart-Bosch A. Cytogenetic analysis of three primary Bellini duct carcinomas. Genes Chromosomes Cancer. 1996;15(3):170–2.PubMedCrossRefGoogle Scholar
  25. 25.
    Schoenberg M, Cairns P, Brooks JD, Marshall FF, Epstein JI, Isaacs WB, et al. Frequent loss of chromosome arms 8p and 13q in collecting duct carcinoma (CDC) of the kidney. Genes Chromosomes Cancer. 1995;12(1):76–80.PubMedCrossRefGoogle Scholar
  26. 26.
    Polascik TJ, Cairns P, Epstein JI, Fuzesi L, Ro JY, Marshall FF, et al. Distal nephron renal tumors: microsatellite allelotype. Cancer Res. 1996;56(8):1892–5.PubMedGoogle Scholar
  27. 27.
    Selli C, Amorosi A, Vona G, Sestini R, Travaglini F, Bartoletti R, et al. Retrospective evaluation of c-erbB-2 oncogene amplification using competitive PCR in collecting duct carcinoma of the kidney. J Urol. 1997;158(1):245–7.PubMedCrossRefGoogle Scholar
  28. 28.
    Rao P, Tannir NM, Tamboli P. Expression of OCT3/4 in renal medullary carcinoma represents a potential diagnostic pitfall. Am J Surg Pathol. 2012;36(4):583–8.PubMedCrossRefGoogle Scholar
  29. 29.
    Swartz MA, Karth J, Schneider DT, Rodriguez R, Beckwith JB, Perlman EJ. Renal medullary carcinoma: clinical, pathologic, immunohistochemical, and genetic analysis with pathogenetic implications. Urology. 2002;60(6):1083–9.PubMedCrossRefGoogle Scholar
  30. 30.
    Liu Q, Galli S, Srinivasan R, Linehan WM, Tsokos M, Merino MJ. Renal medullary carcinoma: molecular, immunohistochemistry, and morphologic correlation. Am J Surg Pathol. 2013;37(3):368–74.PubMedCrossRefGoogle Scholar
  31. 31.
    Cheng JX, Tretiakova M, Gong C, Mandal S, Krausz T, Taxy JB. Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior. Mod Pathol. 2008;21(6):647–52.PubMedCrossRefGoogle Scholar
  32. 32.
    Stahlschmidt J, Cullinane C, Roberts P, Picton SV. Renal medullary carcinoma: prolonged remission with chemotherapy, immunohistochemical characterisation and evidence of bcr/abl rearrangement. Med Pediatr Oncol. 1999;33(6):551–7.PubMedCrossRefGoogle Scholar
  33. 33.
    Schaeffer EM, Guzzo TJ, Furge KA, Netto G, Westphal M, Dykema K, et al. Renal medullary carcinoma: molecular, pathological and clinical evidence for treatment with topoisomerase-inhibiting therapy. BJU Int. 2009;106(1):62–5.PubMedCrossRefGoogle Scholar
  34. 34.
    Albadine R, Wang W, Brownlee NA, Toubaji A, Billis A, Argani P, et al. Topoisomerase II α status in renal medullary carcinoma: immuno-expression and gene copy alterations of a potential target of therapy. J Urol. 2009;182(2):735–40.PubMedCentralPubMedCrossRefGoogle Scholar
  35. 35.
    Motzer RJ, Bacik J, Mariani T, Russo P, Mazumadar M, Reuter V. Treatment outcome and survival associated with metastatic renal cell carcinoma of non-clear-cell histology. J Clin Oncol. 2002;20(9):2376–81.PubMedCrossRefGoogle Scholar
  36. 36.
    Karakiewicz PI, Trinh Q-D, Rioux-Leclercq N, La Taille DA, Novara G, Tostain J, et al. Collecting duct renal cell carcinoma: a matched analysis of 41 cases. Eur Urol. 2007;52(4):1140–6.PubMedCrossRefGoogle Scholar
  37. 37.
    Chao D, Zisman A, Pantuck AJ, Gitlitz BJ, Freedland SJ, Said JW, et al. Collecting duct renal cell carcinoma: clinical study of a rare tumor. J Urol. 2002;167(1):71–4.PubMedCrossRefGoogle Scholar
  38. 38.
    Matsumoto H, Wada T, Aoki A, Hoshii Y, Takahashi M, Aizawa S, et al. Collecting duct carcinoma with long survival treated by partial nephrectomy. Int J Urol. 2001;8(7):401–3.PubMedCrossRefGoogle Scholar
  39. 39.
    Yoshida K, Kinoshita H, Taniguti H, Chizaki R, Nishida T, Hiura Y, et al. Bellini duct carcinoma of the kidney: a case report. Hinyokika Kiyo. 2007;53(2):121–4.PubMedGoogle Scholar
  40. 40.
    Vazquez-Lavista LG, Uribe-Uribe N, Gabilondo-Navarro F. Collecting duct renal cell carcinoma: two different clinical stages, two different clinical outcomes. Urol Int. 2008;81(1):116–8.PubMedCrossRefGoogle Scholar
  41. 41.
    Blom JHM, van Poppel H, Maréchal JM, Jacqmin D, Schröder FH, de Prijck L, et al. Radical nephrectomy with and without lymph-node dissection: final results of European Organization for Research and Treatment of Cancer (EORTC) randomized phase 3 trial 30881. Eur Urol. 2009;55(1):28–34.PubMedCrossRefGoogle Scholar
  42. 42.
    Whitson JM, Harris CR, Reese AC, Meng MV. Lymphadenectomy improves survival of patients with renal cell carcinoma and nodal metastases. J Urol. 2011;185(5):1615–20.PubMedCrossRefGoogle Scholar
  43. 43.
    Sun M, Trinh Q-D, Bianchi M, Hansen J, Abdollah F, Tian Z, et al. Extent of lymphadenectomy does not improve the survival of patients with renal cell carcinoma and nodal metastases: biases associated with the handling of missing data. BJU Int. 2013;113(1):36–42.PubMedCrossRefGoogle Scholar
  44. 44.
    Hellenthal NJ, Mansour AM, Hayn MH, Schwaab T. Is there a role for partial nephrectomy in patients with metastatic renal cell carcinoma? Urol Oncol. 2013;31(1):36–41.PubMedCrossRefGoogle Scholar
  45. 45.
    Mejean A, Roupret M, Larousserie F, Hopirtean V, Thiounn N, Dufour B. Is there a place for radical nephrectomy in the presence of metastatic collecting duct (Bellini) carcinoma? J Urol. 2003;169(4):1287–90.PubMedCrossRefGoogle Scholar
  46. 46.
    Oudard S, Banu E, Vieillefond A, Fournier L, Priou F, Medioni J, 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-Génitales) study. J Urol. 2007;177(5):1698–702.PubMedCrossRefGoogle Scholar
  47. 47.
    Bagrodia A, Gold R, Handorf C, Liman A, Derweesh IH. Salvage paclitaxel chemotherapy for metastatic collecting duct carcinoma of the kidney. Can J Urol. 2008;15(6):4425–7.PubMedGoogle Scholar
  48. 48.
    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.PubMedCrossRefGoogle Scholar
  49. 49.
    Fyfe G, Fisher RI, Rosenberg SA, Sznol M, Parkinson DR, Louie AC. Results of treatment of 255 patients with metastatic renal cell carcinoma who received high-dose recombinant interleukin-2 therapy. J Clin Oncol. 1995;13(3):688–96.PubMedGoogle Scholar
  50. 50.
    Pyrhönen S, Salminen E, Ruutu M, Lehtonen T, Nurmi M, Tammela T, et al. Prospective randomized trial of interferon alfa-2a plus vinblastine versus vinblastine alone in patients with advanced renal cell cancer. J Clin Oncol. 1999;17(9):2859–67.PubMedGoogle Scholar
  51. 51.
    Upton MP, Parker RA, Youmans A, McDermott DF, Atkins MB. Histologic predictors of renal cell carcinoma response to interleukin-2-based therapy. J Immunother. 2005;28(5):488–95.PubMedCrossRefGoogle Scholar
  52. 52.
    Motzer RJ, Rini BI, Bukowski RM, Curti BD, George DJ, Hudes GR, et al. Sunitinib in patients with metastatic renal cell carcinoma. JAMA. 2006;295(21):2516–24.PubMedCrossRefGoogle Scholar
  53. 53.
    Escudier B, Eisen T, Stadler WM, Szczylik C, Oudard S, Siebels M, et al. Sorafenib in advanced clear-cell renal cell carcinoma. N Engl J Med. 2007;356(2):125–34.PubMedCrossRefGoogle Scholar
  54. 54.
    Hudes G, Carducci M, Tomczak P, Dutcher J, Figlin R, Kapoor A, et al. Temsirolimus, interferon alfa, or both for advanced renal cell carcinoma. N Engl J Med. 2007;356(22):2271–81.PubMedCrossRefGoogle Scholar
  55. 55.
    Motzer RJ, Escudier B, Oudard S, Hutson TE, Porta C, Bracarda S, et al. Efficacy of everolimus in advanced renal cell carcinoma: a double-blind, randomised, placebo-controlled phase III trial. Lancet. 2008;372(9637):449–56.PubMedCrossRefGoogle Scholar
  56. 56.
    Sternberg CN, Davis ID, Mardiak J, Szczylik C, Lee E, Wagstaff J, et al. Pazopanib in locally advanced or metastatic renal cell carcinoma: results of a randomized phase III trial. J Clin Oncol. 2010;28(6):1061–8.PubMedCrossRefGoogle Scholar
  57. 57.
    Tannir NM, Plimack E, Ng C, Tamboli P, Bekele NB, Xiao L, et al. A phase 2 trial of sunitinib in patients with advanced non–clear cell renal cell carcinoma. Eur Urol. 2012;62(6):1013–9.PubMedCrossRefGoogle Scholar
  58. 58.
    Staehler M, Schöppler G, Haseke N, Stadler T, Karl A, Siebels M, et al. Carcinoma of the collecting ducts of Bellini of the kidney: adjuvant chemotherapy followed by multikinase inhibition with sunitinib. Clin Genitourin Cancer. 2009;7(1):58–61.PubMedCrossRefGoogle Scholar
  59. 59.
    Miyake H, Haraguchi T, Takenaka A, Fujisawa M. Metastatic collecting duct carcinoma of the kidney responded to sunitinib. Int J Clin Oncol. 2010;16(2):153–5.PubMedCrossRefGoogle Scholar
  60. 60.
    Ansari J, Fatima A, Chaudhri S, Bhatt RI, Wallace M, James ND. Sorafenib induces therapeutic response in a patient with metastatic collecting duct carcinoma of kidney. Onkologie. 2009;32(1–2):44–6.PubMedCrossRefGoogle Scholar
  61. 61.
    Koh Y, Lim HY, Ahn JH, Lee JL, Rha SY, Kim YJ, et al. Phase II trial of everolimus for the treatment of nonclear-cell renal cell carcinoma. Ann Oncol. 2013;24(4):1026–31.PubMedCrossRefGoogle Scholar
  62. 62.
    Procopio G, Verzoni E, Iacovelli R, Colecchia M, Torelli T, Mariani L. Is there a role for targeted therapies in the collecting ducts of Bellini carcinoma? Efficacy data from a retrospective analysis of 7 cases. Clin Exp Nephrol. 2012;16(3):464–7.PubMedCrossRefGoogle Scholar
  63. 63.
    Voss MH, Bastos DA, Karlo CA, Ajeti A, Hakimi AA, Feldman DR, et al. Treatment outcome with mTOR inhibitors for metastatic renal cell carcinoma with nonclear and sarcomatoid histologies. Ann Oncol. 2014;25(3):663–8.PubMedCentralPubMedCrossRefGoogle Scholar
  64. 64.
    Hakimi AA, Koi PT, Milhoua PM, Blitman NM, Li M, Hugec V, et al. Renal medullary carcinoma: the Bronx experience. Urology. 2007;70(5):878–82.PubMedCrossRefGoogle Scholar
  65. 65.
    Avery RA, Harris JE, Davis CJ, Borgaonkar DS. Renal medullary carcinoma: clinical and therapeutic aspects of a newly described tumor. Cancer. 1996;78(1):128–32.PubMedCrossRefGoogle Scholar
  66. 66.
    Walsh A, Kelly DR, Vaid YN, Hilliard LM, Friedman GK. Complete response to carboplatin, gemcitabine, and paclitaxel in a patient with advanced metastatic renal medullary carcinoma. Pediatr Blood Cancer. 2010;55(6):1217–20.PubMedCrossRefGoogle Scholar
  67. 67.
    Rathmell WK, Monk JP. High-dose-intensity MVAC for advanced renal medullary carcinoma: report of three cases and literature review. Urology. 2008;72(3):659–63.PubMedCrossRefGoogle Scholar
  68. 68.
    Kalyanpur A, Schwartz DS, Fields JM. Renal medulla carcinoma in a white adolescent. AJR Am J Roentgenol. 1997;169(4):1037–8.PubMedCrossRefGoogle Scholar
  69. 69.
    Coogan CL, McKiel Jr CF, Flanagan MJ, Bormes TP, Matkov TG. Renal medullary carcinoma in patients with sickle cell trait. Urology. 1998;51(6):1049–50.PubMedCrossRefGoogle Scholar
  70. 70.
    Pirich LM, Chou P, Walterhouse DO. Prolonged survival of a patient with sickle cell trait and metastatic renal medullary carcinoma. J Pediatr Hematol Oncol. 1999;21(1):67.PubMedCrossRefGoogle Scholar
  71. 71.
    Warren KE, Gidvani-Diaz V, Duval-Arnould B. Renal medullary carcinoma in an adolescent with sickle cell trait. Pediatrics. 1999;103(2), e22.PubMedCrossRefGoogle Scholar
  72. 72.
    Figenshau RS, Basler JW, RItter JH, Siegel CL, Simon JA, Dierks SM. Renal medullary carcinoma. J Urol. 1998;159(3):711–3.PubMedCrossRefGoogle Scholar
  73. 73.
    Yang XJ, Sugimura J, Tretiakova MS, Furge K, Zagaja G, Sokoloff M, et al. Gene expression profiling of renal medullary carcinoma. Cancer. 2004;100(5):976–85.PubMedCrossRefGoogle Scholar
  74. 74.
    Strouse JJ, Spevak M, Mack AK, Arceci RJ, Small D, Loeb DM. Significant responses to platinum-based chemotherapy in renal medullary carcinoma. Pediatr Blood Cancer. 2005;44(4):407–11.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  • Jamie Koo
    • 1
  • Christopher P. Filson
    • 2
  • Jiaoti Huang
    • 1
  • Allan J. Pantuck
    • 2
    Email author
  1. 1.Department of Pathology and Laboratory MedicineDavid Geffen School of Medicine at UCLALos AngelesUSA
  2. 2.Department of UrologyInstitute of Urologic Oncology, David Geffen School of Medicine at UCLALos AngelesUSA

Personalised recommendations