Skip to main content
SpringerLink
Log in

Merkel Cell Carcinoma

  • Chapter
Skin Cancer after Organ Transplantation

Part of the book series: Cancer Treatment and Research ((CTAR,volume 146))

Merkel cell carcinoma (MCC) is a rare but aggressive skin cancer, first described by Toker in 1972 as “trabecular carcinoma of the skin” [1]. It is also known as primary neuroendocrine skin carcinoma, Toker tumor, cutaneous apudoma, or Merkel cell tumor. The origin of this malignancy from epidermal Merkel cells is likely but not unequivocally proven, and therefore the term primary neuroendocrine skin carcinoma seems more appropriate; however, the term Merkel cell carcinoma has prevailed in the literature and is adopted here. To date, several hundreds of MCC cases have been reported. An important percentage of these develop in immunocompromised patients, including solid organ transplant recipients (OTR). The salient features of MCC, with emphasis on those cases appearing in the setting of organ transplantation, are reviewed here.

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 169.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 249.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
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. Toker C. Trabecular carcinoma of the skin. Arch Dermatol 1972; 105:107–10.

    Article  PubMed  CAS  Google Scholar 

  2. Hodgson N. Merkel cell carcinoma: changing incidence trends. J Surg Oncol 2005; 89:1–4.

    Article  PubMed  Google Scholar 

  3. Medina-Franco H, Urist M, Fiveash J, et al. Multimodality treatment of Merkel cell carcinoma: case series and literature review of 24 cases. Ann Surg Oncol 2001; 8:204–8.

    Article  PubMed  CAS  Google Scholar 

  4. Agelli M, Clegg LX. Epidemiology of Merkel cell carcinoma in the United States. J Am Acad Dermatol 2003; 49:832–41.

    Article  PubMed  Google Scholar 

  5. Stempfle H, Mudra H, Angermann C, et al. Rapid growth of cutaneous neuroendocrine (Merkel cell) carcinoma during treatment of refractory cardiac allograft rejection with OKT3 monoclonal antibody. J Heart Lung Transplant 1993; 12:501–3.

    PubMed  CAS  Google Scholar 

  6. Formica M, Basolo B, Funaro L, et al. Merkel cell carcinoma in renal transplant recipient. Nephron 1994; 68:399.

    Article  PubMed  CAS  Google Scholar 

  7. Douds A, Mellotte G, Morgan H. Fatal Merkel-cell tumour (cutaneous neuroendocrine carcinoma) complicating renal transplantation. Nephrol Dial Transplant 1995; 10:2346–8.

    PubMed  CAS  Google Scholar 

  8. Vazquez-Mazariego Y, Vallcorba I, Ferro M, et al. Cytogenetic study of neuroendocrine carcinoma of Merkel cells. Cancer Genet Cytogenet 1996; 92:79–81.

    Article  PubMed  CAS  Google Scholar 

  9. Gooptu C, Woolons A, Ross J, et al. Merkel cell carcinoma arising after therapeutic immunosuppression. Br J Dermatol 1997; 137:637–41.

    Article  PubMed  CAS  Google Scholar 

  10. Jonas S, Rayes N, Neumann U, et al. De novo malignancies after liver transplantation using tacrolimus-based protocols or cyclosporine-based quadruple immunosuppression with an interleukin-2 receptor antibody or antithymocyte globulin. Cancer 1997; 80:1141–50.

    Article  PubMed  CAS  Google Scholar 

  11. Williams R, Morgan M, Mathieson I, et al. Merkel cell carcinoma in a renal transplant patient: increased incidence? Transplantation 1998; 27:1396–97.

    Article  Google Scholar 

  12. Plunkett T, Harris A, Ogg C, et al. The treatment of Merkel cell carcinoma and its association with immunosuppression. Br J Dermatol 1998; 139:345–6.

    Article  PubMed  CAS  Google Scholar 

  13. Urbatsch A, Sams M, Urist M, et al. Merkel cell carcinoma occurring in renal transplant recipients. J Am Acad Dermatol 1999; 41:289–91.

    Article  PubMed  CAS  Google Scholar 

  14. Traest K, De Vos R, van den Oord J. Pagetoid Merkel cell carcinoma: speculations on its origin and the mechanism of epidermal spread. J Cutan Pathol 1999; 26:362–5.

    Article  PubMed  CAS  Google Scholar 

  15. Silvestris N, D’Elia F, Tarantino G, et al. Merkel cell carcinoma in a renal transplant patient. J Exp Clin Cancer Res 2000; 19:399–400.

    PubMed  CAS  Google Scholar 

  16. Picciotto F, Basolo B, Massara C, Aloi F, Segoloni G, Quarello F. Tumori cutanei rari in pazienti con trapianto d’organo: nostra esperienzia personale e revisione della letteratura. Giorn Ital Dermatol Venereol 2000; 135:597–605.

    Google Scholar 

  17. Walsh N. Primary neuroendocrine (Merkel cell) carcinoma of the skin: morphologic diversity and implications thereof. Hum Pathol 2001; 32:680–9.

    Article  PubMed  CAS  Google Scholar 

  18. Baron J, Sanchez C, Garcia P. Merkel cell carcinoma in a kidney transplant patient. Rev Clin Esp 2001; 201:223.

    PubMed  CAS  Google Scholar 

  19. Friedlaender M, Rubinger D, Rosenbaum E, et al. Temporary regression of Merkel cell carcinoma metastases after cessation of cyclosporine. Transplantation 2002; 73:1849–50.

    Article  PubMed  Google Scholar 

  20. Maksimyuk B, Wygoda A, Zakliczynski M, et al. Primary undifferentiated small cell carcinoma (Merkel cell carcinoma) in a patient after heart transplantation – a case report. Pol J Pathol 2002; 53:177–80.

    Google Scholar 

  21. Buell JF, Trofe J, Hanaway MJ, et al. Immunosuppression and Merkel cell cancer. Transplant Proc 2002; 34:1780–1.

    Article  PubMed  CAS  Google Scholar 

  22. Bartsch C, Mechtersheimer G, Helmchen B, et al. Merkel-Zell-Karzinom nach Nierentransplantation. Pathologe 2002; 23:308–12.

    Article  PubMed  CAS  Google Scholar 

  23. Clark B, Jukic D, Lourduraj L. Case 398-Multiple cutaneous malignancies and related fatal outcome. Available at: http://path.upmc.edu/cases/case398.html. Accessed April 2007.

  24. Morris K, Williams B, Kennedy G. Heavy bone marrow involvement with metastatic Merkel cell tumour in an immunosuppressed renal transplant recipient. Br J Haematol 2005; 128:133.

    Article  PubMed  CAS  Google Scholar 

  25. Esen B, Pinarbasi B, Buyukbabani N, et al. Merkel-cell carcinoma arising after liver transplantation: a case report. Transplant Proc 2005; 37:4413–5.

    Article  PubMed  CAS  Google Scholar 

  26. Ferreira P, Pinho C, Cardoso A, et al. Unusual Merkel cell carcinoma in a renal transplant recipient: case report and review of the literature. Eur J Plast Surg 2006; 28:426–31.

    Article  Google Scholar 

  27. Kanitakis J, Euvrard S, Chouvet B, Butnaru AC, Claudy A. Merkel cell carcinoma in renal transplant recipients. Report of two new cases with unusual histological features and literature review. J Cutan Pathol 2006; 33:686–94.

    Article  PubMed  Google Scholar 

  28. Muirhead R, Ritchie D. Partial regression of Merkel cell carcinoma in response to withdrawal of azathioprine in an immunosuppression-induced case of metastatic Merkel cell carcinoma. Clin Oncol 2007; 19:96.

    Article  CAS  Google Scholar 

  29. Bensaleh H, Perney P, Dereure O, et al. Merkel cell carcinoma in a liver transplant patient. Am J Clin Dermatol 2007; 8:239–41.

    Article  PubMed  Google Scholar 

  30. Bajetta E, Platania M, Catena L, et al. Merkel cell carcinoma after liver transplantation: a case report. Tumori 2007; 93:323–6.

    PubMed  Google Scholar 

  31. Dantal J, Hourmant M, Cantarovitch D, Mansat E, et al. Effect of long-term immunosuppression in kidney-graft recipients on cancer incidence: randomised comparison of two cyclosporine regimens. Lancet 1998; 351:623–8.

    Article  PubMed  CAS  Google Scholar 

  32. Bordea C, Wojnarowska F, Millard P, et al. Skin cancer in renal-transplant recipients occur more frequently than previously recognized in a temperate climate. Transplantation 2004; 77:574–9.

    Article  PubMed  CAS  Google Scholar 

  33. Penn I, First MR. Merkel’s cell carcinoma in organ recipients: report of 41 cases. Transplantation 1999; 68:1717–21.

    Article  PubMed  CAS  Google Scholar 

  34. Miller R, Rabkin C. Merkel cell carcinoma and melanoma: etiological similarities and differences. Cancer Epidemiol Biomarkers Prev 1999; 8:153–8.

    PubMed  CAS  Google Scholar 

  35. Mojica P, Smith D, Ellenhorn J. Adjuvant radiation therapy is associated with improved survival in Merkel cell carcinoma of the skin. J Clin Oncol 2007; 25:1043–7.

    Article  PubMed  Google Scholar 

  36. Popp S, Waltering S, Herbst C, et al. UV-B-type mutations and chromosomal imbalances indicate common pathways for the development of Merkel and skin squamous cell carcinomas. Int J Cancer 2002; 99:352–60.

    Article  PubMed  CAS  Google Scholar 

  37. Matichard E, Descamps V, Grossin M, et al. Merkel cell carcinoma in a black human immunodeficiency virus-infected patient. Br J Dermatol 2002; 146:671–3.

    Article  PubMed  CAS  Google Scholar 

  38. Vlad R, Woodlock T. Merkel cell carcinoma after chronic lymphatic leukaemia: case report and literature review. Am J Clin Oncol 2003; 26:531–4.

    Article  PubMed  Google Scholar 

  39. Euvrard S, Kanitakis J, Claudy A. Skin cancers after organ transplantation. N Engl J Med 2003; 348:1681–91.

    Article  PubMed  Google Scholar 

  40. Kanitakis J, Joly MO, Chouvet B, et al. Merkel cell carcinomas in immunocompetent and organ-transplant recipients do not harbor HPV. Eur J Dermatol 2006; 16:445–6.

    PubMed  Google Scholar 

  41. Shaw M, Warren S, Groben P, Gulley M. No evidence of Epstein–Barr virus association with Merkel cell carcinoma. J Cutan Pathol 2006; 33:624–8.

    Article  PubMed  Google Scholar 

  42. Feng H, Shuda M, Chang Y, Moore P. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science 2008; 319:1096–100.

    Article  PubMed  CAS  Google Scholar 

  43. Ho SY, Tsai YC, Lee YY, et al. Merkel cell carcinoma in patients with long-term ingestion of arcenic. J Occup Health 2005; 47:188–92.

    Article  PubMed  Google Scholar 

  44. Brenn T, McKee P. Neuroendocrine skin carcinoma. In: McKee P, Calonje E, Granter S (eds) Pathology of the Skin with Clinical Correlations, 3rd edn. Philadelphia: Elsevier Mosby, 2005:1230–8.

    Google Scholar 

  45. Rocamora A, Badia N, Vives R, et al. Epidermotropic primary neuroendocrine (Merkel cell) carcinoma of the skin with Pautrier-like microabscesses. Report of three cases and review of the literature. J Am Acad Dermatol 1987; 16:1163.

    Article  PubMed  CAS  Google Scholar 

  46. Brown H, Sawyer D, Woo T. Intraepidermal Merkel cell carcinoma with no dermal involvement. Am J Dermatopathol 2000; 22:65–9.

    Article  PubMed  CAS  Google Scholar 

  47. Llombart B, Monteagudo C, Lopez-Guerrero J, et al. Clinicopathological and immunohistochemical analysis of 20 cases of Merkel cell carcinoma in search of prognostic markers. Histopathology 2005; 46:622–34.

    Article  PubMed  CAS  Google Scholar 

  48. Skelton H, Smith K, Hitchkock C, et al. Merkel cell carcinoma: analysis of clinical, histologic, and immunohistologic features of 132 cases with relation to survival. J Am Acad Dermatol 1997; 37:734–9.

    Article  PubMed  CAS  Google Scholar 

  49. Goldberg S, Neifeld J, Frable W. Prognostic value of tumor thickness in patients with Merkel cell carcinoma. J Surg Oncol 2007; 95:618–22 (doi 10.1002/jso.20737).

    Article  PubMed  Google Scholar 

  50. Kanitakis J. Solid cutaneous tumours. In: Kanitakis J, Vassileva S, Woodley D (eds) Diagnostic Immunohistochemistry of the Skin. An Illustrated Text. London: Chapman & Hall, 1998:279–99.

    Google Scholar 

  51. Vesely M, Murray D, Neligan P, et al. Complete spontaneous regression in Merkel cell carcinoma. J Plast Reconstr Aesth Surg 2008; 61:165–71.

    Article  Google Scholar 

  52. Gupta S, Wang L, Penas P, et al. Sentinel lymph node biopsy for evaluation and treatment of patients with Merkel cell carcinoma: the Dana-Farber experience and meta-analysis of the literature. Arch Dermatol 2006; 142:685–90.

    Article  PubMed  Google Scholar 

  53. Berg D, Otley C. Skin cancer in organ transplant recipients: epidemiology, pathogenesis, and management. J Am Acad Dermatol 2002; 47:1–17.

    Article  PubMed  Google Scholar 

  54. Veness M, Harris D. Role of radiotherapy in the management of organ transplant recipients diagnosed with non-melanoma skin cancers. Australas Radiol 2007; 51:12–20.

    Article  PubMed  CAS  Google Scholar 

  55. Lewis K, Weinstock M, Weaver A, Otley C. Adjuvant local irradiation for Merkel cell carcinoma. Arch Dermatol 2006; 142:693–700.

    Article  PubMed  Google Scholar 

  56. Boyer J, Zitelli J, Broadland D, et al. Local control of primary Merkel cell carcinoma: review of 45 cases treated with Mohs micrographic surgery with and without adjuvant radiation. J Am Acad Dermatol 2002; 47:885–92.

    Article  PubMed  Google Scholar 

  57. Senchenkov A, Barnes S, Moran S. Predictors of survival and recurrence in the surgical treatment of Merkel cell carcinoma of the extremities. J Surg Oncol 2007; 95:229–34.

    Article  PubMed  Google Scholar 

  58. Clark B, Veness M, Gilbert R, et al. Merkel cell carcinoma of the head and neck: is adjuvant radiotherapy necessary? Head Neck 2007; 29:249–57.

    Article  PubMed  Google Scholar 

  59. Jabbour J, Cumming R, Scolyer RA, et al. Merkel cell carcinoma: assessing the effect of wide local excision, lymph node dissection, and radiotherapy on recurrence and survival in early-stage disease: results from a review of 82 consecutive cases diagnosed between 1992 and 2004. Ann Surg Oncol 2007; 14: 1943–52.

    Article  PubMed  Google Scholar 

  60. Brady M. Current management of patients with Merkel cell carcinoma. Dermatol Surg 2004; 30:321–5.

    Article  PubMed  Google Scholar 

  61. Tai P, Yu E, Winquist E, et al. Chemotherapy in neuroendocrine/Merkel cell carcinoma of the skin: case series and review of 204 cases. J Clin Oncol 2000; 18:2493–99.

    PubMed  CAS  Google Scholar 

  62. Swick B, Ravdel L, Fitzpatrick J, et al. Merkel cell carcinoma: evaluation of KIT (CD117) expression and failure to demonstrate activating mutations in the c-KIT protooncogene – implications for treatment with imatinib mesylate. J Cutan Pathol 2007; 34:324–9.

    Article  PubMed  Google Scholar 

  63. Campistol J, Eris J, Oberbauer R, et al. Sirolimus therapy after early cyclosporine withdrawal reduces the risk for cancer in adult renal transplantation. J Am Soc Nephrol 2006; 17:581–9.

    Article  PubMed  CAS  Google Scholar 

  64. Guba M, von Breitenbuch P, Steinbauer M, et al. Rapamycin inhibits primary and metastatic tumor growth by antiangiogenesis: involvement of vascular endothelial growth factor. Nat Med 2002; 8:128–35.

    Article  PubMed  CAS  Google Scholar 

  65. Euvrard S, Ulrich C, Lefrançois N. Immunosuppressants and skin cancer in transplant patients: focus on rapamycin. Dermatol Surg 2004; 30(4 pt 2): 628–33.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Dermatology, Edouard Herriot Hospital, 60437 Lyon Cedex 03, France

    Jean Kanitakis

Authors
  1. Jean Kanitakis
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Klinik für Dermatologie Venerologie und Allergologie, Charité - Universitätsklinikum Berlin Charite Campus Mitte Klinik und Poliklinik for Dermatologie, Charitéplatz 1, 10117 Berlin, Germany

    Eggert Stockfleth MD, PhD  & Claas Ulrich  & 

Rights and permissions

Reprints and permissions

Copyright information

© 2009 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Kanitakis, J. (2009). Merkel Cell Carcinoma. In: Stockfleth, E., Ulrich, C. (eds) Skin Cancer after Organ Transplantation. Cancer Treatment and Research, vol 146. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-78574-5_27

Download citation

  • DOI: https://doi.org/10.1007/978-0-387-78574-5_27

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-0-387-78573-8

  • Online ISBN: 978-0-387-78574-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation