title: Merkel cell carcinoma. Diagnosis and treatment.
abstract: BACKGROUND
  Merkel cell carcinoma is an uncommon malignancy of the skin that often portends a poor prognosis. Since its first description by Toker in 1972, a plethora of case reports and articles regarding the etiopathogenesis and treatment have been published spanning multiple medical and surgical disciplines. Much confusion still exists regarding the diagnosis and treatment of this ominous tumor.
  
  
  OBJECTIVE
  Through extensive review of the medical, surgical, and pathological literature, to collate the observations of multiple investigators and summarize these findings.
  
  
  METHODS
  Articles from journals of multiple subspecialties were carefully reviewed with particular emphasis placed on epidemiology, prognosis, histology, immunohistochemistry, electron microscopy, tumor origin, treatment, and work-up of Merkel cell carcinoma.
  
  
  RESULTS
  Merkel cell carcinoma is an aggressive malignant neoplasm. Local recurrence develops in 26-44% of patients despite therapy. Up to three-fourths of patients eventually develop regional nodal metastases with distant metastases occurring in one-third of all patients. Reported overall 5-year survival rates range from 30% to 64%.
  
  
  CONCLUSIONS
  Treatment recommendations unfortunately are based more on anecdotal than scientific data because of the rarity of the tumor and its recognized high risk. Most authors recommend wide local excision of the primary lesion and regional lymph node resection if lymph nodes are palpable followed by x-irradiation of both the postsurgical bed and lymph node basin. The role of elective lymph node resection in the absence of clinically positive nodes remains controversial.
authors.affiliation: Division of Dermatology and Cutaneous Surgery, University of South Florida College of Medicine, Tampa 33612, USA.
authors.forename: M L
authors.initials: ML
authors.lastname: Haag
authors.forename: L F
authors.initials: LF
authors.lastname: Glass
authors.forename: N A
authors.initials: NA
authors.lastname: Fenske
mesh_terms: D015266:Carcinoma, Merkel Cell / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D006801:Humans; D012878:Skin Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy