title: Merkel cell carcinoma: report of 10 cases and review of the literature.
abstract: BACKGROUND
  Merkel cell carcinoma (MCC) is a rare primary neuroendocrine skin tumor that usually arises in the head and neck or the extremities of elderly patients. Because of the limitation of retrospective data, optimal treatment is not well defined.
  
  
  OBJECTIVE
  Our purpose was to present the clinical course and treatment of 10 patients with MCC and review the published literature on MCC.
  
  
  METHODS
  We conducted a retrospective analysis and obtained detailed clinical information for all 10 patients treated for MCC at our institution from 1986 through 1998. The medical literature was also reviewed for natural history and treatment recommendations using MEDLINE search.
  
  
  RESULTS
  Five men and 5 women received their treatment between 1986 and 1998 for MCC (5 had stage IA disease, 4 stage IB, 1 stage II). The mean age was 70.3 years (range, 47-86 years). Seven tumors were located on the head and neck and 3 on extremities. Five of 10 patients had a relapse (mean time before recurrence, 5.7 months) (range, 2 weeks-20 months); one patient had local recurrence, one had regional lymph node recurrence, and 3 had both local and regional lymph node recurrence. In 4 patients systemic metastases developed. Long survival is also noted (6 to > 164 months); 4 patients died of MCC. After initial surgery, 9 patients received radiotherapy at some point and 3 patients also received chemotherapy. Five of 10 patients had 13 previously treated or coexisting malignant neoplasms. In one patient MCC developed in a previously irradiated field. Review of 875 cases showed a male/female ratio of 1.5:1; location of tumors was as follows: head and neck, 47%; extremities, 40%; trunk, 8%; unknown primary site, 5%. Local recurrence was observed in 25%, regional lymph node involvement in 52%, distant metastasis in 34%, and MCC was a cause of death in 34%.
  
  
  CONCLUSIONS
  MCC has a high incidence of locoregional recurrence with distant metastases that is more common with higher stage lesions. Early local management of smaller lesions results in good long-term survival. It is not known whether prophylactic lymph node dissection and/or radiation and adjuvant radiation increases survival. Long survival can be achieved after treating locoregional recurrence. The role of chemotherapy is still controversial and should be considered in patients with advanced disease and those not thought to be candidates for surgery.
authors.affiliation: Department of Medicine, Division of Medical Hematology and Oncology, University Hospital, State University of New York, Health Science Center at Syracuse, USA. akhtars18@hotmail.com
authors.forename: S
authors.initials: S
authors.lastname: Akhtar
authors.forename: K K
authors.initials: KK
authors.lastname: Oza
authors.forename: J
authors.initials: J
authors.lastname: Wright
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015266:Carcinoma, Merkel Cell / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D017024:Chemotherapy, Adjuvant; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012878:Skin Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D016019:Survival Analysis; D016896:Treatment Outcome