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Melanoma pp 623-636 | Cite as

Management of Non-melanoma Skin Cancers: Merkel Cell Carcinoma

  • Isabel Prieto
  • Teresa Pérez-de-la-Fuente
  • M Susana Medina
  • Beatriz Castelo
  • Fernando Cassinello
  • David Esteban
  • Nuria Rodriguez-Salas
Chapter

Abstract

Merkel cell carcinoma is a rare primary cutaneous carcinoma of the skin, which shows high aggressiveness, high risk of locoregional and distant spread, a mortality rate considerably higher than that of cutaneous melanoma, and a poor survival. Its incidence has increased during the past 20 years. The studies published from 2008 have introduced interesting changes in the understanding of its epidemiology, pathogenesis and consequently in the diagnostic codes and the therapeutic options. These advancements in our understanding of Merkel cell carcinoma have contributed to better defining the high-risk patients, which may require a more comprehensive approach to treatment. A multidisciplinary team approach to the overall management is crucial for the optimal treatment of patients with Merkel cell carcinoma. Surgery and radiotherapy play a fundamental role in the management of this tumor, with both associated with improved locoregional control and disease-free survival. The risk for systemic recurrence is quite high, with no truly validated prognostic factors to identify those patients who will experience a distant failure. For those that develop metastatic disease, adjuvant chemotherapy can be utilized with little benefit toward a longer survival. Newer treatment options examining the role of immunotherapy have shown great promise in ongoing clinical trials and will likely replace chemotherapy as the preferred treatment option for stage 4 Merkel cell carcinoma in the future.

Keywords

Merkel cell carcinoma Polyomavirus Head and neck Merkel cell carcinoma Sentinel lymph node biopsy Immunotherapy Targeted therapies Biomarkers 

Abbreviations

CLND

Complete lymph node dissection

CRT

Chemoradiotherapy

CTR

Clinical trials

DFS

Disease-free survival

H&N

Head and neck

IT

Immunotherapy

LTAg

Large tumor antigen

MCC

Merkel cell carcinoma

MCV

Merkel cell polyomavirus

NAFT

Nuclear factor of activated T cells

NK

Natural killer

OS

Overall survival

PD-1

Death protein-1

PD-L1

PD-1 ligand

RT

Radiation therapy

SEER

Surveillance, Epidemiology, and End Results

SLNB

Sentinel lymphatic node biopsy

sTAg

Small tumor antigen

UV

Ultraviolet light

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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Isabel Prieto
    • 1
  • Teresa Pérez-de-la-Fuente
    • 2
  • M Susana Medina
    • 3
  • Beatriz Castelo
    • 4
  • Fernando Cassinello
    • 5
  • David Esteban
    • 1
  • Nuria Rodriguez-Salas
    • 4
  1. 1.Department of Radiation OncologyFundación Jiménez DíazMadridSpain
  2. 2.Department of Plastic SurgeryFundación Jiménez DíazMadridSpain
  3. 3.Department of DermatologyPríncipe de Asturias University HospitalMadridSpain
  4. 4.Department of Medical OncologyHospital Universitario La PazMadridSpain
  5. 5.Department of AnesthesiologyFundación Jiménez DíazMadridSpain

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