title: Clinical management and treatment outcomes of Merkel cell carcinoma.
abstract: BACKGROUND
  Merkel cell carcinoma (MCC), first described in 1972, is an uncommon, highly malignant tumour of skin. Its aetiology is unknown although the tumour occurs most frequently in sun-exposed sites. This skin cancer is characterized by a high incidence of metastases, local and regional recurrence and has a high mortality. Few survival data beyond 3 years are available. Treatment strategies include wide local excision of the primary and lymph node dissection, as indicated together with adjuvant radiotherapy. The tumour has also been shown to be chemosensitive. Definitive treatment is inconclusive due to the relative rarity of this malignancy.
  
  
  METHODS
  A retrospective study was undertaken between the years 1985 and 1996 of 33 patients with MCC at Concord Repatriation General Hospital (CRGH). Demographic details were noted, together with the site and appearance of the primary lesion and the sites and date of metastases. The response and outcome to various modalities of treatment were documented.
  
  
  RESULTS
  There were 27 men and six women with an average age of 80 years (range: 60-86 years). The primary lesion in the present series most often resembled a basal cell carcinoma (BCC), with 53% occurring in the head and neck region. Twenty-seven patients (82%) developed metastatic disease, with an average interval of 13.4 months between diagnosis of the primary lesion and the development of metastases. The incidence of locoregional recurrence in the present series was 42%. Radiation combined with surgery achieved locoregional control in 15 of 19 patients with primary, regional or recurrent locoregional disease. Fourteen patients died of MCC and five others from unrelated causes. The 14 survivors have a mean survival of 54 months with six patients surviving more than 5 years.
  
  
  CONCLUSIONS
  Local excision together with regional lymph node clearance as indicated, combined with adjuvant radiation treatment was associated with an improved survival. The role of chemotherapy remains unclear.
authors.affiliation: Concord Repatriation General Hospital, Sydney, New South Wales, Australia.
authors.forename: K C
authors.initials: KC
authors.lastname: Wong
authors.forename: F
authors.initials: F
authors.lastname: Zuletta
authors.forename: S J
authors.initials: SJ
authors.lastname: Clarke
authors.forename: P J
authors.initials: PJ
authors.lastname: Kennedy
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015266:Carcinoma, Merkel Cell / Q000401:mortality / Q000556:secondary / Q000601:surgery*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D015994:Incidence; D008197:Lymph Node Excision; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D015996:Survival Rate; D016896:Treatment Outcome