Pediatric melanoma in melanoma‐prone families. Issue 18 (12th September 2018)

• Record Type: Journal Article
• Authors: Goldstein, Alisa M.; Stidd, Kelsey C.; Yang, Xiaohong R.; Fraser, Mary C.; Tucker, Margaret A.
• Is Part Of: Cancer. Volume 124:Issue 18(2018)
• Page Start: 3715
• Page End: 3723
• Publication Date: 2018-09-12
• Subjects: cyclin‐dependent kinase inhibitor 2A (CDKN2A) -- family research -- genetics -- melanoma -- pediatrics -- sun protection; Cancer -- Periodicals; Cancer -- Cytopathology -- Periodicals; 616.99405
• DOI: 10.1002/cncr.31641
• Languages: English
• ISSNs: 0008-543X

Abstract

Abstract : 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. Abstract : Regardless of cyclin‐dependent kinase inhibitor 2A ( CDKN2A ) status, melanoma‐prone families are reported 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. Children in melanoma‐prone families should have careful sun protection from an early age and skin surveillance to reduce their risk of melanoma.