High-risk surveillance for patients with Li-Fraumeni syndrome (LFS) has shown a stage shift and improved overall survival, but is demanding. Our objective was to evaluate surveillance adherence in a population of patients with LFS presenting for high-risk care. A retrospective analysis of surveillance adherence of adult patients with LFS at a single institution was performed. Adherence was defined by the duration from initial University of Virginia (UVA) LFS clinic visit to the time of first missed surveillance test. Two-sample t-tests and ANOVA tests were used to identify factors associated with duration of adherence. A total of 42 patients were evaluated in the UVA LFS clinic between 2017 and 2021. Of these, 21 patients met inclusion criteria. At the time of review, 6 patients (29%) were up to date with high-risk surveillance recommendations. The mean duration of adherence was 17 months. Female sex was found to be associated with longer duration of adherence (mean 21 mo vs. 3.5 mo for males, p = 0.02). A personal history or active diagnosis of cancer was also associated with increased adherence (p = 0.02). However, neither age (p = 0.89), geography (p = 0.84), or known family history of LFS (p = 0.08) were associated with duration of adherence. Female sex as well as a personal history of cancer were associated with longer duration of adherence to recommended high-risk surveillance among patients with LFS. Identification of barriers to surveillance will be essential moving forward to increase adherence and promote early detection of cancer, thereby reducing the morbidity and mortality of LFS.

中文翻译：

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影响 Li-Fraumeni 综合征患者遵守高风险监测方案的因素

对李法美尼综合征 (LFS) 患者的高风险监测已显示出阶段性转变并改善了总体生存率，但要求很高。我们的目标是评估接受高风险护理的 LFS 患者群体的监测依从性。对单一机构 LFS 成年患者的监测依从性进行了回顾性分析。依从性的定义是从初次就诊弗吉尼亚大学 (UVA) LFS 诊所到首次错过监测测试的时间的持续时间。使用双样本 t 检验和方差分析检验来确定与坚持持续时间相关的因素。2017 年至 2021 年间，UVA LFS 诊所总共对 42 名患者进行了评估。其中，21 名患者符合纳入标准。截至审查时，6 名患者 (29%) 已了解最新的高风险监测建议。平均坚持时间为 17 个月。研究发现，女性的依从时间较长（平均 21 个月，男性为 3.5 个月，p = 0.02）。癌症的个人病史或主动诊断也与依从性的增加相关（p = 0.02）。然而，年龄 (p = 0.89)、地理位置 (p = 0.84) 或已知的 LFS 家族史 (p = 0.08) 与坚持持续时间无关。LFS 患者中女性以及个人癌症史与坚持建议的高风险监测时间较长有关。确定监测障碍对于提高依从性和促进癌症的早期发现至关重要，从而降低 LFS 的发病率和死亡率。