Merkel Cell Carcinoma Clinical Practice Guidelines in OncologyNCCN Categories of Evidence and ConsensusCategory 1: The recommendation is based on high-level evidence (e.g., randomized controlled trials) and there is uniform NCCN consensus.Category 2A: The recommendation is based on lower-level evidence and there is uniform NCCN consensus.Category 2B: The recommendation is based on lower-level evidence and there is nonuniform NCCN consensus (but no major disagreement).Category 3: The recommendation is based on any level of evidence but reflects major disagreement.All recommendations are category 2A unless otherwise noted.Clinical trials: The NCCN believes that the best management for any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged.OverviewMerkel cell carcinoma (MCC) is a rare, aggressive cutaneous tumor that combines the local recurrence rates of infiltrative non-melanoma skin cancer along with the regional and distant metastatic rates of thick melanoma.1–16 Several large reviews document the development of local recurrence in 25% to 30% of all cases of MCC, regional disease in 52% to 59%, and distant metastatic disease in 34% to 36%.1,16,17 MCC has a mortality rate that exceeds that of melanoma;18 overall 5-year survival rates range from 30% to 64%.3,19 A history of extensive sun exposure is a risk factor for MCC. Older white men (≥ 65 years) are at higher risk for MCC, which tends to occur on the areas of the skin that are exposed to sun.20The NCCN Non-Melanoma Skin Cancer Panel has developed guidelines outlining treatment of...

If the inline PDF is not rendering correctly, you can download the PDF file here.

References

  • 1

    AkhtarSOzaKKWrightJ. Merkel cell carcinoma: report of 10 cases and review of the literature. J Am Acad Dermatol2000;43:755767.

  • 2

    AllenPJZhangZFCoitDG. Surgical management of Merkel cell carcinoma. Ann Surg1999;229:97105.

  • 3

    BichakjianCKLoweLLaoCD. Merkel cell carcinoma: critical review with guidelines for multidisciplinary management. Cancer2007;110:112.

    • Search Google Scholar
    • Export Citation
  • 4

    GoesslingWMcKeePHMayerRJ. Merkel cell carcinoma. J Clin Oncol2002;20:588598.

  • 5

    GruberSBWilsonLD. Merkel cell carcinoma. In: Cutaneous Oncology: Pathophysiology Diagnosis and Management. Malden (MA):Blackwell Science; 1998:710721.

    • Search Google Scholar
    • Export Citation
  • 6

    HaagMLGlassLFFenskeNA. Merkel cell carcinoma. Diagnosis and treatment. Dermatol Surg1995;21:669683.

  • 7

    HitchcockCLBlandKILaneyRGIII. Neuroendocrine (Merkel cell) carcinoma of the skin. Its natural history, diagnosis, and treatment. Ann Surg1988;207:201207.

    • Search Google Scholar
    • Export Citation
  • 8

    KokoskaERKokoskaMSCollinsBT. Early aggressive treatment for Merkel cell carcinoma improves outcome. Am J Surg1997;174:688693.

  • 9

    LawendaBDThiringerJKFossRDJohnstonePA. Merkel cell carcinoma arising in the head and neck: optimizing therapy. Am J Clin Oncol2001;24:3542.

    • Search Google Scholar
    • Export Citation
  • 10

    OttMJTanabeKKGaddMA. Multimodality management of Merkel cell carcinoma. Arch Surg1999;134:388392; discussion 392–383.

  • 11

    PitaleMSessionsRBHusainS. An analysis of prognostic factors in cutaneous neuroendocrine carcinoma. Laryngoscope1992;102:244249.

  • 12

    PoulsenM. Merkel-cell carcinoma of the skin. Lancet Oncol2004;5:593599.

  • 13

    RatnerDNelsonBRBrownMDJohnsonTM. Merkel cell carcinoma. J Am Acad Dermatol1993;29:143156.

  • 14

    SkeltonHGSmithKJHitchcockCL. Merkel cell carcinoma: analysis of clinical, histologic, and immunohistologic features of 132 cases with relation to survival. J Am Acad Dermatol1997;37:734739.

    • Search Google Scholar
    • Export Citation
  • 15

    WongKCZulettaFClarkeSJKennedyPJ. Clinical management and treatment outcomes of Merkel cell carcinoma. Aust N Z J Surg1998;68:354358.

    • Search Google Scholar
    • Export Citation
  • 16

    Medina-FrancoHUristMMFiveashJ. Multimodality treatment of Merkel cell carcinoma: case series and literature review of 1024 cases. Ann Surg Oncol2001;8:204208.

    • Search Google Scholar
    • Export Citation
  • 17

    GillenwaterAMHesselACMorrisonWH. Merkel cell carcinoma of the head and neck: effect of surgical excision and radiation on recurrence and survival. Arch Otolaryngol Head Neck Surg2001;127:149154.

    • Search Google Scholar
    • Export Citation
  • 18

    NghiemPMcKeePHaynesHA. Merkel cell (cutaneous neuroendocrine) carcinoma. In: SoberAJHaluskaFG eds. Skin Cancer. Hamilton (Ontario): BC Decker Inc.; 2001:127141.

    • Search Google Scholar
    • Export Citation
  • 19

    AllenPJBowneWBJaquesDP. Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J Clin Oncol2005;23:23002309.

    • Search Google Scholar
    • Export Citation
  • 20

    AgelliMCleggLX. Epidemiology of primary Merkel cell carcinoma in the United States. J Am Acad Dermatol2003;49:832841.

  • 21

    MillerSJAlamMAndersenJ. NCCN Clinical Practice Guidelines in Oncology: Merkel Cell Carcinoma. J Natl Compr Canc Netw2006;4:704712.

  • 22

    CheukWKwanMYSusterSChanJK. Immunostaining for thyroid transcription factor 1 and cytokeratin 20 aids the distinction of small cell carcinoma from Merkel cell carcinoma, but not pulmonary from extrapulmonary small cell carcinomas. Arch Pathol Lab Med2001;125:228231.

    • Search Google Scholar
    • Export Citation
  • 23

    HanlyAJElgartGWJordaM. Analysis of thyroid transcription factor-1 and cytokeratin 20 separates merkel cell carcinoma from small cell carcinoma of lung. J Cutan Pathol2000;27:118120.

    • Search Google Scholar
    • Export Citation
  • 24

    ScottMPHelmKF. Cytokeratin 20: a marker for diagnosing Merkel cell carcinoma. Am J Dermatopathol1999;21:1620.

  • 25

    FeinmesserMHalpernMKaganovskyE. C-KIT Expression in primary and metastatic merkel cell carcinoma. Am J Dermatopathol2004;26:458462.

  • 26

    AllenPBusamKHillAD. Immunohistochemical analysis of sentinel lymph nodes from patients with Merkel cell carcinoma. Cancer2001;92:16501655.

    • Search Google Scholar
    • Export Citation
  • 27

    SchmalbachCELoweLTeknosTN. Reliability of sentinel lymph node biopsy for regional staging of head and neck Merkel cell carcinoma. Arch Otolaryngol Head Neck Surg2005;131:610614.

    • Search Google Scholar
    • Export Citation
  • 28

    SuLDLoweLBradfordCR. Immunostaining for cytokeratin 20 improves detection of micrometastatic Merkel cell carcinoma in sentinel lymph nodes. J Am Acad Dermatol2002;46:661666.

    • Search Google Scholar
    • Export Citation
  • 29

    GreeneFLPageDLFlemingID. AJCC CancerStaging Manual6th ed. AJCC Springer-Verlag: New York; 2002.

  • 30

    O’ConnorWJRoenigkRKBrodlandDG. Merkel cell carcinoma. Comparison of Mohs micrographic surgery and wide excision in eighty-six patients. Dermatol Surg1997;23:929933.

    • Search Google Scholar
    • Export Citation
  • 31

    PenningtonBELeffellDJ. Mohs micrographic surgery: established uses and emerging trends. Oncology (Williston Park)2005;19:11651171.

  • 32

    GuptaSGWangLCPenasPF. 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 Dermatol2006;142:685690.

    • Search Google Scholar
    • Export Citation
  • 33

    AmesSEKragDNBradyMS. Radiolocalization of the sentinel lymph node in Merkel cell carcinoma: a clinical analysis of seven cases. J Surg Oncol1998;67:251254.

    • Search Google Scholar
    • Export Citation
  • 34

    HillADBradyMSCoitDG. Intraoperative lymphatic mapping and sentinel lymph node biopsy for Merkel cell carcinoma. Br J Surg1999;86:518521.

    • Search Google Scholar
    • Export Citation
  • 35

    MehranyKOtleyCCWeenigRH. A meta-analysis of the prognostic significance of sentinel lymph node status in Merkel cell carcinoma. Dermatol Surg2002;28:113117; discussion 117.

    • Search Google Scholar
    • Export Citation
  • 36

    MessinaJLReintgenDSCruseCW. Selective lymphadenectomy in patients with Merkel cell (cutaneous neuroendocrine) carcinoma. Ann Surg Oncol1997;4:389395.

    • Search Google Scholar
    • Export Citation
  • 37

    LewisKGWeinstockMAWeaverALOtleyCC. Adjuvant local irradiation for Merkel cell carcinoma. Arch Dermatol2006;142:693700.

  • 38

    JabbourJCummingRScolyerRA. 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 Oncol2007;14:19431952.

    • Search Google Scholar
    • Export Citation
  • 39

    GarneskiKMNghiemP. Merkel cell carcinoma adjuvant therapy: current data support radiation but not chemotherapy. J Am Acad Dermatol2007;57:166169.

    • Search Google Scholar
    • Export Citation
  • 40

    TaiP. Merkel cell cancer: update on biology and treatment. Curr Opin Oncol2008;20:196200.

  • 41

    PoulsenMRischinDWalpoleE. High-risk Merkel cell carcinoma of the skin treated with synchronous carboplatin/etoposide and radiation: a Trans-Tasman Radiation Oncology Group Study—TROG 96:07. J Clin Oncol2003;21:43714376.

    • Search Google Scholar
    • Export Citation
  • 42

    PoulsenMGRischinDPorterI. Does chemotherapy improve survival in high-risk stage I and II Merkel cell carcinoma of the skin?Int J Radiat Oncol Biol Phys2006;64:114119.

    • Search Google Scholar
    • Export Citation
  • 43

    TaiPTYuEWinquistE. Chemotherapy in neuroendocrine/Merkel cell carcinoma of the skin: case series and review of 204 cases. J Clin Oncol2000;18:24932499.

    • Search Google Scholar
    • Export Citation
  • 44

    VoogEBironPMartinJPBlayJY. Chemotherapy for patients with locally advanced or metastatic Merkel cell carcinoma. Cancer1999;85:25892595.

    • Search Google Scholar
    • Export Citation

Article Information

PubMed

Google Scholar

Related Articles

Metrics

All Time Past Year Past 30 Days
Abstract Views 0 0 0
Full Text Views 64 64 12
PDF Downloads 21 21 7
EPUB Downloads 0 0 0