title: An evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis.
abstract: BACKGROUND
  Familial cylindromatosis is a rare genetic disorder, giving rise to neoplasms of the skin appendages. We have recently shown that loss of the cylindromatosis tumour suppressor gene leads to activation of NF-kappaB, a transcription factor having antiapoptotic activity. This provides a possible explanation for the deregulated growth of cylindromas. In cell-based assays, salicylate can prevent NF-kappaB activation caused by loss of the cylindromatosis gene, suggesting that salicylic acid application might be a potential treatment for cylindromatosis.
  
  
  OBJECTIVE
  To assess the effectiveness of topical application of salicylic acid on familial cylindromas.
  
  
  METHODS
  Cylindromas in five patients from four different cylindromatosis families were treated with twice daily and then once daily topical salicylic acid. Clinical response was determined by serial tumour measurements.
  
  
  RESULTS
  In total 17 cylindromas in five patients were studied: 12 target lesions and five control lesions. The median size of the cylindromas was 1.0 cm (range, 0.6-2.8 cm). Two of the 12 cylindromas showed a complete remission. Another eight lesions showed some response, but not sufficient to qualify as partial remission. The control lesions remained stable or increased in size.
  
  
  CONCLUSIONS
  Salicylic acid is a well-tolerated and potential new treatment for cylindromatosis.
authors.affiliation: Division of Molecular Carcinogenesis and Center for Biomedical Genetics, The Netherlands Cancer Institute, Amsterdam, The Netherlands.
authors.forename: H M
authors.initials: HM
authors.lastname: Oosterkamp
authors.forename: H
authors.initials: H
authors.lastname: Neering
authors.forename: S M B
authors.initials: SM
authors.lastname: Nijman
authors.forename: A M G
authors.initials: AM
authors.lastname: Dirac
authors.forename: W J
authors.initials: WJ
authors.lastname: Mooi
authors.forename: R
authors.initials: R
authors.lastname: Bernards
authors.forename: T R
authors.initials: TR
authors.lastname: Brummelkamp
mesh_terms: D000287:Administration, Topical; D003528:Carcinoma, Adenoid Cystic / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism; D018280:Carcinoma, Skin Appendage / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D016147:Genes, Tumor Suppressor; D006801:Humans; D051550:I-kappa B Kinase / Q000378:metabolism; D007641:Keratolytic Agents / Q000008:administration & dosage* / Q000627:therapeutic use; D016328:NF-kappa B / Q000378:metabolism; D009386:Neoplastic Syndromes, Hereditary / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism; D010865:Pilot Projects; D012074:Remission Induction; D020156:Salicylic Acid / Q000008:administration & dosage* / Q000627:therapeutic use
chemical_list: D007641:Keratolytic Agents; D016328:NF-kappa B; D051550:I-kappa B Kinase; D020156:Salicylic Acid