title: Effectiveness and causes for failure of surveillance of CDKN2A-mutated melanoma families.
abstract: BACKGROUND
  For more than 25 years families with an increased susceptibility to melanoma have been under surveillance at our institution.
  
  
  OBJECTIVE
  We sought to investigate the effectiveness of surveillance for CDKN2A-mutated families and causes for failure of the program in patients with more advanced tumors.
  
  
  METHODS
  In a retrospective case-control study, Breslow thickness of melanomas diagnosed in relatives enrolled in the surveillance program were compared with melanomas of unscreened index patients. We investigated the influence of mode of detection and length of surveillance interval on outcome.
  
  
  RESULTS
  Surveillance melanomas (n = 226, median thickness: 0.50 mm) had a significantly lower Breslow thickness (multiplication factor: 0.61 [95% confidence interval 0.47-0.80], P < .001) than index melanomas (n = 40, median thickness: 0.98 mm). Index melanomas were more likely diagnosed with a Breslow thickness greater than 1.0 mm (odds ratio: 3.1 [95% confidence interval 1.2-8.1], P = .022). In all, 53% of surveillance melanomas were diagnosed during regular screens, 7% during patients' first screen, 20% between regular screens, and 20% in patients who were noncompliant with the surveillance schedule. The majority of surveillance melanomas (58%) were detected within 6 months after the last screen. There was no correlation between tumor thickness and the length of the screening interval for tumors diagnosed within 24 months since the last screen.
  
  
  CONCLUSIONS
  The study is retrospective.
  
  
  CONCLUSIONS
  Surveillance was associated with earlier detection of melanomas. Noncompliance was an important cause for failing surveillance. Shortening surveillance intervals may advance detection of tumors, but may paradoxically have little impact on prognosis.
authors.affiliation: Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands. Electronic address: j.i.van_der_rhee@lumc.nl.
authors.forename: Jasper I
authors.initials: JI
authors.lastname: van der Rhee
authors.affiliation: Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands; Department of Human and Clinical Genetics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Femke A
authors.initials: FA
authors.lastname: de Snoo
authors.affiliation: The Netherlands Foundation for the Detection of Hereditary Tumors, Leiden, The Netherlands.
authors.forename: Hans F A
authors.initials: HFA
authors.lastname: Vasen
authors.affiliation: Department of Pathology, Vrije Universiteit University Medical Center, Amsterdam, The Netherlands.
authors.forename: Wolter J
authors.initials: WJ
authors.lastname: Mooi
authors.affiliation: Department of Medical Statistics, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Hein
authors.initials: H
authors.lastname: Putter
authors.affiliation: Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Nelleke A
authors.initials: NA
authors.lastname: Gruis
authors.affiliation: Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Nicole A
authors.initials: NA
authors.lastname: Kukutsch
authors.affiliation: Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: Wilma
authors.initials: W
authors.lastname: Bergman
mesh_terms: D017677:Age Distribution; D001707:Biopsy, Needle; D016022:Case-Control Studies; D016001:Confidence Intervals; D055088:Early Detection of Cancer; D005260:Female; D019942:Genes, p16; D020022:Genetic Predisposition to Disease / Q000453:epidemiology*; D018095:Germ-Line Mutation; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D008297:Male; D008403:Mass Screening / Q000458:organization & administration*; D008545:Melanoma / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D009367:Neoplasm Staging; D016017:Odds Ratio; D010375:Pedigree; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012680:Sensitivity and Specificity; D017678:Sex Distribution; D012878:Skin Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D016019:Survival Analysis