title: Treatment of Merkel cell carcinoma of the head and neck: a systematic review.
abstract: BACKGROUND
  Merkel cell carcinoma (MCC) is a rare and aggressive neurocutaneous malignancy that frequently arises in sun-exposed areas of the head and neck. Standard therapy focuses on wide local excision (WLE) with adjuvant locoregional radiotherapy. However, treatment is often complicated by concerns for cosmesis and for preservation of the head and neck neurovasculature.
  
  
  OBJECTIVE
  To explore treatment-related outcomes of the head and neck MCC.
  
  
  METHODS
  A MEDLINE and Google Scholar search was performed for studies focusing on the head and neck MCC treatment.
  
  
  RESULTS
  The search terms produced 100 articles. Seventeen studies met eligibility/screening criteria, yielding 868 patients. Three of the 6 relevant studies found a significant difference in disease-free survival (DFS) between surgery and surgery plus adjuvant radiation. Two studies found no difference in DFS or overall survival (OS) in patients receiving chemotherapy. Two studies found no difference in DFS between radiotherapy and surgery with adjuvant radiation. No difference in OS was found between WLE and Mohs surgery.
  
  
  CONCLUSIONS
  In an uncomplicated head and neck MCC, treatment with surgery and adjuvant radiotherapy is effective in increasing survival and reducing recurrence. Radiotherapy alone may be appropriate for inoperable regions. Primary chemotherapy seems to have a limited role; however, few studies explored this treatment modality.
authors.affiliation: *Division of Otolaryngology, Department of Surgery, University of California, San Diego, California; †Division of Dermatology, Department of Medicine, University of California, San Diego, California; ‡Pediatric and Adolescent Dermatology, Rady Children's Hospital, University of California, San Diego, California.
authors.forename: Sharat
authors.initials: S
authors.lastname: Raju
authors.forename: Aria
authors.initials: A
authors.lastname: Vazirnia
authors.forename: Christine
authors.initials: C
authors.lastname: Totri
authors.forename: Tissa R
authors.initials: TR
authors.lastname: Hata
mesh_terms: D015266:Carcinoma, Merkel Cell / Q000628:therapy*; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D006258:Head and Neck Neoplasms / Q000628:therapy*; D006801:Humans; D009364:Neoplasm Recurrence, Local; D011379:Prognosis