title: Noncutaneous Melanomas: A Single-Center Analysis.
abstract: BACKGROUND
  The optimal treatment algorithm for noncutaneous melanomas must yet be established.
  
  
  OBJECTIVE
  To compare systemic treatment-relevant mutational status, metastatic pattern and response to systemic treatment in noncutaneous melanoma.
  
  
  METHODS
  Retrospective single-center study analyzing 64 noncutaneous melanoma patients treated between January 2006 and September 2013.
  
  
  RESULTS
  c-KIT mutations were found exclusively in vulvovaginal melanoma (4/7). Overall status for NRAS and BRAF mutations was low (1/7 and 0/21 detected mutations, respectively). Seven out of 7 vulvovaginal and 6/13 sinonasal melanomas first metastasized to lymph nodes, whereas 18/22 ocular melanomas first metastasized to the liver. Response to systemic treatment in vulvovaginal melanomas was best for imatinib with a disease control rate of 3/3 and overall for ipilimumab with a disease control rate of 3/10. Sorafenib was associated with adverse drug reactions (6/13) and poor results.
  
  
  CONCLUSIONS
  Noncutaneous melanomas show few tumor-signaling pathway mutations and distinct metastasization patterns. Immunotherapy induces response rates in mucosal melanoma similar to those in cutaneous melanoma.
authors.affiliation: Department of Dermatology, University Hospital of Zurich, Zurich, Switzerland.
authors.forename: Valerio
authors.initials: V
authors.lastname: Del Prete
authors.forename: Karla
authors.initials: K
authors.lastname: Chaloupka
authors.forename: David
authors.initials: D
authors.lastname: Holzmann
authors.forename: Daniel
authors.initials: D
authors.lastname: Fink
authors.forename: Mitchell
authors.initials: M
authors.lastname: Levesque
authors.forename: Reinhard
authors.initials: R
authors.lastname: Dummer
authors.forename: Simone M
authors.initials: SM
authors.lastname: Goldinger
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005134:Eye Neoplasms / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D005260:Female; D020558:GTP Phosphohydrolases / Q000235:genetics; D005833:Genital Neoplasms, Female / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008297:Male; D008545:Melanoma / Q000235:genetics* / Q000556:secondary / Q000628:therapy*; D008565:Membrane Proteins / Q000235:genetics; D008875:Middle Aged; D009154:Mutation / Q000235:genetics*; D009669:Nose Neoplasms / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D012189:Retrospective Studies
chemical_list: D008565:Membrane Proteins; D019009:Proto-Oncogene Proteins c-kit; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf; D020558:GTP Phosphohydrolases; C579846:NRAS protein, human