The familial dysplastic nevus syndrome: Natural history and the impact of screening on prognosis a study of nine families in the Netherlands |
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| Institution: | 1. Foundation for the Detection of Hereditary Tumours, Utrecht ,The Netherlands;2. Department of Dermatology, Leiden University Hospital,The Netherlands;3. Clinical Genetics Centre, Leiden University Hospital,The Netherlands;4. Department of Pathology, Leiden University Hospital,The Netherlands;1. Medicinal Chemistry Department, Faculty of Pharmacy (Boys), Al-Azhar University, Cairo 11884, Egypt;2. Medicinal Chemistry Department, Faculty of Pharmacy, Mansoura University, Mansoura 35516, Egypt;3. Biochemistry Department, Faculty of Medicine, King Abdulaziz University, Jeddah 21589, Saudi Arabia;4. Department of Pharmaceutical Chemistry, Faculty of Pharmacy, King Abdulaziz University, Jeddah 21589, Saudi Arabia;5. Pharmacology Department, Faculty of Pharmacy, King Abdulaziz University, Jeddah 21589, Saudi Arabia;1. Department of Entomology, Cornell University, Ithaca, NY 14853, USA;1. National Institute for Health Research (NIHR) Nottingham Hearing Biomedical Research Unit, Ropewalk House, 113 The Ropewalk, Nottingham, NG1 5DU, United Kingdom;2. Otology and Hearing Group, Division of Clinical Neuroscience, School of Medicine, University of Nottingham, Nottingham, NG7 2UH, United Kingdom;3. Institut Arthur Vernes, ENT Surgery, Paris, 75006, France;4. Nottingham University Hospitals NHS Trust, Derby Road, Nottingham, NG7 2UH, United Kingdom;5. Medical Research Council (MRC) Institute of Hearing Research, The University of Nottingham, University Park, Nottingham, NG7 2RD, United Kingdom |
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| Abstract: | Since 1982, nine families with the dysplastic nevus syndrome have been identified in the Leiden area (The Netherlands). A total of 50 primary melanomas were diagnosed in 38 persons. Nineteen of these melanomas had been diagnosed before the start of the screening programme (category I), 11 were detected at the initial examination of the families (category II), and 20 were found during the course of follow-up (category III).To assess the effect of screening, we compared these categories with respect to the developmental stage of the melanomas. One of the 19 melanomas in category I, two of the 11 in category II and seven of the 20 in category III were melanoma in situ. The average thickness of the invasive melanomas in categories I, II and III was 1.75, 0.80 and 0.54 mm respectively. Sixteen of the 19 melanomas in category I (84%) were Clark III or IV, whereas 15 of the 20 melanomas in category III (75%) were Clark I or II.From these findings it may be concluded that screening can lead to the detection of melanomas at an earlier stage, which in turn can permit curative treatment and improvement of both prognosis and life expectancy.The need for supervision based on central registration of affected families to guarantee the continuity of screening is discussed. |
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