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Brief Report
November 30, 2016

Risk Factors for Basal Cell Carcinoma Among Patients With Basal Cell Nevus SyndromeDevelopment of a Basal Cell Nevus Syndrome Patient Registry

Author Affiliations
  • 1Medical student, Stanford University School of Medicine, Stanford, California
  • 2Department of Dermatology, Stanford University School of Medicine, Stanford, California
  • 3Department of Dermatology, MC Zuiderzee Hospital, Lelystad, the Netherlands
  • 4Basal Cell Carcinoma Nevus Syndrome Life Support Network, Burton, Ohio
  • 5Children’s Hospital Oakland Research Institute, Oakland, California
JAMA Dermatol. Published online November 30, 2016. doi:10.1001/jamadermatol.2016.4347
Key Points

Question  What is the burden of basal cell carcinomas (BCCs) in patients with basal cell nevus syndrome, and what factors correlate with higher BCC tumor burden?

Findings  In a clinical registry of 141 patients with basal cell nevus syndrome, participants reported a mean of 257 BCCs over their lifetime. Age and lifetime number of sunburns were significantly associated with higher number of BCCs.

Meaning  Patients with basal cell nevus syndrome have a high burden of BCCs. This clinical registry has identified potential risk factors for BCCs and sets the basis for development of future clinical trials.

Abstract

Importance  Patients with basal cell nevus syndrome (BCNS) have a greater risk of developing numerous basal cell carcinomas (BCCs). Risk factors influencing the wide variation in tumor burden are poorly understood.

Objective  To describe the burden of BCCs in patients with BCNS in the United States and identify potential risk factors for BCCs.

Design, Setting, and Participants  Prospective clinical registry with data collected from September 2014 to March 2016. Participants were recruited from a mailing list of patients with BCNS at Children’s Hospital Oakland Research Institute and Basal Cell Carcinoma Nevus Syndrome Life Support Network. Patients of all ages with a diagnosis of BCNS were eligible for enrollment. Participants completed a clinical questionnaire on their disease characteristics and risk factors.

Main Outcomes and Measures  Number of BCCs in the past 2 years and over lifetime (disease burden), risk factors for BCCs.

Results  A consecutive sample of the first 141 participants was included (34% [100 of 297] response rate from paper survey, 23% [41 of 179] from online survey; 85 [60%] female; mean age at start of study, 53 [range, 8-83] years; 131 [93%] white). In the previous 2 years, participants reported a mean of 25 BCCs (median, 11; range, 0-250). Over their lifetime, participants reported a mean of 257 BCCs (median, 160; range, 0-2200). Univariate analysis identified age (odds ratio [OR], 1.05; 95% CI, 1.03-1.07; P < .001), number of sunburns (OR, 1.05; 95% CI, 1.00-1.10; P = .047), and history of radiation exposure (OR, 2.26; 95% CI, 1.02-5.03; P = .046) as potential risk factors for lifetime BCC severity. On multivariate analysis, only age (OR, 1.04; 95% CI, 1.02-1.07; P < .001) and number of sunburns (OR, 1.06; 95% CI, 1.00-1.11; P = .04) were statistically significant. In our adjusted models, BCC burden increased by 4% per year of age and by 6% per number of sunburns.

Conclusions and Relevance  Patients with BCNS have a high burden of BCCs. Age and number of sunburns were significantly associated with the severity of lifetime BCC. Further interventions to prevent and treat BCCs in patients with BCNS are needed.

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