BACKGROUND: In the United States, only approximately 0.4% of all melanomas are diagnosed in patients aged <20 years. To the authors' knowledge, melanoma in pediatric members of melanoma-prone families has not been fully investigated to date. The objective of the current study was to evaluate pediatric patients with melanoma with extensive follow-up in melanoma-prone families with and without cyclin-dependent kinase inhibitor 2A (CDKN2A) mutations. METHODS: For this non-population-based study, families were followed prospectively for up to 40 years. A total of 60 families with ≥ 3 patients with melanoma were included for analysis: 30 CDKN2A mutation-positive (CDKN2A+) and 30 CDKN2A mutation-negative (CDKN2A-) families. Age at the time of first melanoma and number of melanomas were obtained for each patient and summarized by family or sets (CDKN2A + vs CDKN2A-). For set comparisons and categorical variables (occurrence of melanoma in pediatric patients, number of melanomas, number of patients with single or multiple melanomas), the Pearson chi-square or Fisher exact test was used. RESULTS: Regardless of CDKN2A status, melanoma-prone families were found to have 6-fold to 28-fold higher percentages of patients with pediatric melanoma compared with the general population of patients with melanoma in the United States. Within CDKN2A + families, pediatric patients with melanoma were significantly more likely to have multiple melanomas compared with their relatives who were diagnosed at age >20 years (71% vs 38%, respectively; P = .004). CDKN2A + families had significantly higher percentages of pediatric patients with melanoma compared with CDKN2A- families (11.1% vs 2.5%; P = .004). CONCLUSIONS: These observations have implications for the prevention of melanoma as well as clinical care for its early detection. Children in melanoma-prone families should have careful sun protection from an early age and skin surveillance to reduce their risk of melanoma.
BACKGROUND: In the United States, only approximately 0.4% of all melanomas are diagnosed in patients aged <20 years. To the authors' knowledge, melanoma in pediatric members of melanoma-prone families has not been fully investigated to date. The objective of the current study was to evaluate pediatric patients with melanoma with extensive follow-up in melanoma-prone families with and without cyclin-dependent kinase inhibitor 2A (CDKN2A) mutations. METHODS: For this non-population-based study, families were followed prospectively for up to 40 years. A total of 60 families with ≥ 3 patients with melanoma were included for analysis: 30 CDKN2A mutation-positive (CDKN2A+) and 30 CDKN2A mutation-negative (CDKN2A-) families. Age at the time of first melanoma and number of melanomas were obtained for each patient and summarized by family or sets (CDKN2A + vs CDKN2A-). For set comparisons and categorical variables (occurrence of melanoma in pediatric patients, number of melanomas, number of patients with single or multiple melanomas), the Pearson chi-square or Fisher exact test was used. RESULTS: Regardless of CDKN2A status, melanoma-prone families were found to have 6-fold to 28-fold higher percentages of patients with pediatric melanoma compared with the general population of patients with melanoma in the United States. Within CDKN2A + families, pediatric patients with melanoma were significantly more likely to have multiple melanomas compared with their relatives who were diagnosed at age >20 years (71% vs 38%, respectively; P = .004). CDKN2A + families had significantly higher percentages of pediatric patients with melanoma compared with CDKN2A- families (11.1% vs 2.5%; P = .004). CONCLUSIONS: These observations have implications for the prevention of melanoma as well as clinical care for its early detection. Children in melanoma-prone families should have careful sun protection from an early age and skin surveillance to reduce their risk of melanoma.
Authors: Marianne Berwick; Irene Orlow; Amanda J Hummer; Bruce K Armstrong; Anne Kricker; Loraine D Marrett; Robert C Millikan; Stephen B Gruber; Hoda Anton-Culver; Roberto Zanetti; Richard P Gallagher; Terence Dwyer; Timothy R Rebbeck; Peter A Kanetsky; Klaus Busam; Lynn From; Urvi Mujumdar; Homer Wilcox; Colin B Begg Journal: Cancer Epidemiol Biomarkers Prev Date: 2006-08 Impact factor: 4.254
Authors: Irene Orlow; Colin B Begg; Javier Cotignola; Pampa Roy; Amanda J Hummer; Brian A Clas; Urvi Mujumdar; Rebecca Canchola; Bruce K Armstrong; Anne Kricker; Loraine D Marrett; Robert C Millikan; Stephen B Gruber; Hoda Anton-Culver; Roberto Zanetti; Richard P Gallagher; Terence Dwyer; Timothy R Rebbeck; Peter A Kanetsky; Homer Wilcox; Klaus Busam; Lynn From; Marianne Berwick Journal: J Invest Dermatol Date: 2007-01-11 Impact factor: 8.551
Authors: Alisa M Goldstein; May Chan; Mark Harland; Elizabeth M Gillanders; Nicholas K Hayward; Marie-Francoise Avril; Esther Azizi; Giovanna Bianchi-Scarra; D Timothy Bishop; Brigitte Bressac-de Paillerets; William Bruno; Donato Calista; Lisa A Cannon Albright; Florence Demenais; David E Elder; Paola Ghiorzo; Nelleke A Gruis; Johan Hansson; David Hogg; Elizabeth A Holland; Peter A Kanetsky; Richard F Kefford; Maria Teresa Landi; Julie Lang; Sancy A Leachman; Rona M Mackie; Veronica Magnusson; Graham J Mann; Kristin Niendorf; Julia Newton Bishop; Jane M Palmer; Susana Puig; Joan A Puig-Butille; Femke A de Snoo; Mitchell Stark; Hensin Tsao; Margaret A Tucker; Linda Whitaker; Emanuel Yakobson Journal: Cancer Res Date: 2006-10-15 Impact factor: 12.701
Authors: Victoria K Hill; Jared J Gartner; Yardena Samuels; Alisa M Goldstein Journal: Annu Rev Genomics Hum Genet Date: 2013-07-12 Impact factor: 8.929
Authors: Roy Rabbie; Mamunur Rashid; Ana M Arance; Marcelo Sánchez; Gemma Tell-Marti; Miriam Potrony; Carles Conill; Remco van Doorn; Stefan Dentro; Nelleke A Gruis; Pippa Corrie; Vivek Iyer; Carla Daniela Robles-Espinoza; Joan A Puig-Butille; Susana Puig; David J Adams Journal: Pigment Cell Melanoma Res Date: 2017-04-19 Impact factor: 4.693