Update on Merkel Cell Carcinoma: Epidemiology, Etiopathogenesis, Clinical Features, Diagnosis, and Staging

Actas Dermosifiliogr. 2017 Mar;108(2):108-119. doi: 10.1016/j.ad.2016.07.022. Epub 2016 Oct 19.
[Article in English, Spanish]

Abstract

Merkel cell carcinoma (MCC) is a rare, highly aggressive tumor, and local or regional disease recurrence is common, as is metastasis. MCC usually develops in sun-exposed skin in patients of advanced age. Its incidence has risen 4-fold in recent decades as the population has aged and immunohistochemical techniques have led to more diagnoses. The pathogenesis of MCC remains unclear but UV radiation, immunosuppression, and the presence of Merkel cell polyomavirus in the tumor genome seem to play key roles. This review seeks to update our understanding of the epidemiology, etiology, pathogenesis, and clinical features of MCC. We also review histologic and immunohistochemical features required for diagnosis. MCC staging is discussed, given its great importance in establishing a prognosis for these patients.

Keywords: Carcinoma de células de Merkel; Clinical features; Clínica; Estadificación; Histology; Histología; Immunohistochemistry; Inmunohistoquímica; Merkel cell carcinoma; Poliomavirus; Polyomaviruses; Staging.

Publication types

  • Review

MeSH terms

  • Age of Onset
  • Aged
  • Carcinoma, Merkel Cell* / diagnosis
  • Carcinoma, Merkel Cell* / epidemiology
  • Carcinoma, Merkel Cell* / etiology
  • Carcinoma, Merkel Cell* / pathology
  • Chromosome Aberrations
  • Female
  • Follow-Up Studies
  • Genes, p53
  • Humans
  • Immunohistochemistry
  • Male
  • Merkel cell polyomavirus / pathogenicity
  • Middle Aged
  • Neoplasm Staging
  • Neoplasms, Radiation-Induced / diagnosis
  • Neoplasms, Radiation-Induced / epidemiology
  • Neoplasms, Radiation-Induced / etiology
  • Neoplasms, Radiation-Induced / pathology
  • Polyomavirus Infections / epidemiology
  • Prognosis
  • Risk Factors
  • Skin Neoplasms* / diagnosis
  • Skin Neoplasms* / epidemiology
  • Skin Neoplasms* / etiology
  • Skin Neoplasms* / pathology
  • Sunlight / adverse effects
  • Tumor Virus Infections / epidemiology