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Merkel Cell Carcinoma

  • Sheena Tsai
  • Jeremy S. Bordeaux
Chapter

Abstract

Merkel cell carcinoma (MCC) is a rare, aggressive skin cancer. The incidence has increased over the past 20 years, and this may be attributed to increased ultraviolet (UV) light exposure. MCC is more common among Caucasians, males, and individuals older than 69 years of age. Immunosuppressed patients are at a higher risk for MCC. MCC may present clinically as a dome-shaped red or blue nodule. The mnemonic “AEIOU” describes common clinical features: asymptomatic, expanding rapidly, immunosuppression, older than 50 years, and ultraviolet-exposed/fair skin. MCC most commonly presents on the head and neck, followed by the extremities and trunk. Diagnosis is made via biopsy, and the first-line treatment is surgical removal of the primary tumor. Sentinel lymph node biopsy (SLNB) is recommended for all patients: patients with negative sentinel lymph nodes (SLNs) may undergo wide local excision (WLE), Mohs micrographic surgery (MMS), or radiation to the primary tumor site. Patients with positive SLNs may undergo WLE or MMS, followed by complete lymph node dissection (CLND) and/or radiation to the nodal basin. Patients with metastatic disease may undergo chemotherapy or immunotherapy. Prognosis for MCC patients depends on the stage of disease: survival rates decrease as the stage advances. Due to the high recurrence rate of MCC, patients are encouraged to see their dermatologists frequently.

Keywords

Merkel cell carcinoma Skin cancer Epidemiology Clinical features Diagnosis Histopathology Staging Treatment Prognosis 

References

  1. 1.
    Smith KR. The ultrastructure of the human Haarscheibe and Merkel cell. J Invest Dermatol. 1970;54:150–9.CrossRefPubMedGoogle Scholar
  2. 2.
    Hashimoto K. The ultrastructure of the skin of human embryos. X. Merkel tactile cells in the finger and nail. J Anat. 1972;111:99–120.PubMedPubMedCentralGoogle Scholar
  3. 3.
    Kurosumi K, Kurosumi U, Suzuki H. Fine structures of Merkel cells and associated nerve fibers in the epidermis of certain mammalian species. Arch Histol Jpn. 1969;30:295–313.CrossRefPubMedGoogle Scholar
  4. 4.
    Halata Z, Grim M, Bauman KI. Friedrich Sigmund Merkel and his “Merkel cell”, morphology, development, and physiology: review and new results. Anat Rec. 2003;271:225–39.CrossRefGoogle Scholar
  5. 5.
    Iggo A, Muir AR. The structure and function of a slowly adapting touch corpuscle in hairy skin. J Physiol. 1969;200:763–96.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Lemos B, Nghiem P. J Investig Dermatol. 2007;127:2100.CrossRefPubMedGoogle Scholar
  7. 7.
    Feng H, Shuda M, Chang Y, Moore PS. Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science. 2008;319(5866):1096–100.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Miller RW, Rabkin CS. Merkel cell carcinoma and melanoma: etiological similarities and differences. Cancer Epidemiol Biomark Prev. 1999;8:153–8.Google Scholar
  9. 9.
    Hussain SK, Sundquist J, Hemminki K. Incidence trends of squamous cell and rare skin cancers in the Swedish national cancer registry point to calendar year and age-dependent increases. J Invest Dermatol. 2010;130:1323–8.CrossRefPubMedGoogle Scholar
  10. 10.
    Medina-Franco H, Urist MM, Fiveash J, Heslin MJ, et al. Multimodality treatment of Merkel cell carcinoma: case series and literature review of 1024 cases. Ann Surg Oncol. 2001;8:204–8.CrossRefPubMedGoogle Scholar
  11. 11.
    Key statistics for Merkel cell carcinoma [Internet]. American Cancer Society. 2016 [cited 12 December 2016]. Available from: http://www.cancer.org/cancer/skincancer-merkelcell/detailedguide/skin-cancer-merkel-cell-carcinoma-key-statistics.
  12. 12.
    Tadmor T, Aviv A, Polliack A. Merkel cell carcinoma, chronic lymphocytic leukemia and other lymphoproliferative disorders: an old bond with possible new viral ties. Ann Oncol. 2011;22:250–6.CrossRefPubMedGoogle Scholar
  13. 13.
    Duprat JP, Landman G, Salvajoli JV, Brechtbühl ER. A review of the epidemiology and treatment of Merkel cell carcinoma. Clinics (Sao Paulo). 2011;66(10):1817–23.CrossRefGoogle Scholar
  14. 14.
    Wang TS, Byrne PJ, Jacobs LK, Taube JM. Merkel cell carcinoma: update and review. Semin Cutan Med Surg. 2011;30:48–56.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Heath M, Jaimes N, Lemos B, et al. Clinical characteristics of Merkel cell carcinoma at diagnosis in 195 patients: the AEIOU features. J Am Acad Dermatol. 2008;58:375–81.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Kim DK, Holbrook KA. The appearance, density and distribution of Merkel cells in human embryonic and fetal skin: their relation to sweat gland and hair follicle development. J Invest Dermatol. 1995;104(3):411–416.CrossRefPubMedGoogle Scholar
  17. 17.
    Eispert AC, Fuchs F, Brandner JM, et al. Evidence for distinct populations of human Merkel cells. Histochem Cell Biol. 2009;132:83–93.CrossRefPubMedGoogle Scholar
  18. 18.
    Moll R, Löwe A, Cytokeratin LJ. 20 in human carcinomas. A new histodiagnostic marker detected by monoclonal antibodies. Am J Pathol. 1992;140:427–47.PubMedPubMedCentralGoogle Scholar
  19. 19.
    Hill AD, Brady MS, Coit DG. Intraoperative lymphatic mapping and sentinel lymph node biopsy for Merkel cell carcinoma. Br J Surg. 1999;86(4):518–21.CrossRefPubMedGoogle Scholar
  20. 20.
    Messina JL, Reintgen DS, Cruse CW, et al. Selective lymphadenectomy in patients with Merkel cell (cutaneous neuroendocrine) carcinoma. Ann Surg Oncol. 1997;4(5):389–95.CrossRefPubMedGoogle Scholar
  21. 21.
    Edge S, Greene FL, Byrd DR, Brookland RK, et al., editors. AJCC cancer staging manual. 8th ed. New York: Springer; 2017.Google Scholar
  22. 22.
    Overview of Mohs Micrographic Surgery: Mohs Surgery Patient Education [Internet]. Skincancermohssurgery.org. 2016 [cited 13 December 2016]. Available from: http://www.skincancermohssurgery.org/about-mohs-surgery/overview-of-mohs-micrographic-surgery.
  23. 23.
    Lewis KG, Weinstock MA, Weaver AL, et al. Adjuvant local irradiation for Merkel cell carcinoma. JAMA Dermatol. 2006;142:693–700.Google Scholar
  24. 24.
    Mojica P, Smith D, Ellenhorn JD. Adjuvant radiation therapy is associated with improved survival in Merkel cell carcinoma of the skin. J Clin Oncol. 2007;25:1043–7.CrossRefPubMedGoogle Scholar
  25. 25.
    Voog E, Biron P, Martin JP, et al. Chemotherapy for patients with locally advanced or metastatic Merkel cell carcinoma. Cancer. 1999;85:2589–95.CrossRefPubMedGoogle Scholar
  26. 26.
    Lipson EJ, Vincent JG, Loyo M, Kagohara LT, et al. PD-L1 expression in the Merkel cell microenvironment association with inflammation, Merkel cell polyomavirus and overall survival. Cancer Immunol Res. 2013;1:10.CrossRefPubMedCentralGoogle Scholar
  27. 27.
    Walocko FM, Scheier BY, Harms PW, et al. Metastatic Merkel cell carcinoma response to nivolumab. J Immunother Cancer. 2016;4:79.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Nghiem PT, Bhatia S, Lipson EJ, et al. PD-1 blockade with pembrolizumab in advanced Merkel-cell carcinoma. N Engl J Med. 2016;374:2542–52.CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Kaufman HL, Russell J, Hamid O, Bhatia S, et al. Avelumab in patients with chemotherapy refractory metastatic Merkel cell carcinoma: a multicenter, single-group, open-label, phase 2 trial. Lancet Oncol. 2016;17(10):1374–85.CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Survival rates for Merkel cell carcinoma, by stage [Internet]. American Cancer Society. 2016 [cited 12 December 2016]. Available from: http://www.cancer.org/cancer/skincancer-merkelcell/detailedguide/skin-cancer-merkel-cell-carcinoma-survival-rates.

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.University Hospitals Cleveland Medical Center and Case Western Reserve University School of MedicineClevelandUSA

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