Late morbidity and mortality in adult survivors of childhood glioma with neurofibromatosis type 1: report from the Childhood Cancer Survivor Study.,Genetics in Medicine

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Late morbidity and mortality in adult survivors of childhood glioma with neurofibromatosis type 1: report from the Childhood Cancer Survivor Study.
Genetics in Medicine ( IF 6.6 ) Pub Date : 2020-06-23 , DOI: 10.1038/s41436-020-0873-7
Peter de Blank ₁ , Nan Li ₂ , Michael J Fisher ₃ , Nicole J Ullrich ₄ , Smita Bhatia ₅ , Yutaka Yasui ₂ , Charles A Sklar ₆ , Wendy Leisenring ₇ , Rebecca Howell ₈ , Kevin Oeffinger ₉ , Kristina Hardy ₁₀ , M Fatih Okcu ₁₁ , Todd M Gibson ₂ , Leslie L Robison ₂ , Gregory T Armstrong ₂ , Kevin R Krull ₂

Affiliation

University of Cincinnati and Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Departments of Epidemiology and Cancer Control, Oncology and Psychology, St. Jude Children's Research Hospital, Memphis, TN, USA.
University of Pennsylvania Perlman School of Medicine, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Harvard Medical School, Boston Children's Hospital, Boston, MA, USA.
Institute for Cancer Outcomes and Survivorship, University of Alabama at Birmingham, Birmingham, AL, USA.
Memorial Sloan Kettering Cancer Center, New York, NY, USA.
Cancer Prevention and Clinical Statistics Programs, Fred Hutchinson Cancer Research Center, Seattle, WA, USA.
Department of Radiation Physics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Department of Medicine, Duke University, Durham, NC, USA.
Children's National Medical Center, Washington, DC, USA.
Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA.

Purpose

Neurofibromatosis type 1 (NF1) is associated with tumor predisposition and nonmalignant health conditions. Whether survivors of childhood cancer with NF1 are at increased risk for poor long-term health outcomes is unknown.

Methods

One hundred forty-seven 5+ year survivors of childhood glioma with NF1 from the Childhood Cancer Survivor Study were compared with 2629 non-NF1 glioma survivors and 5051 siblings for late mortality, chronic health conditions, and psychosocial, neurocognitive, and socioeconomic outcomes.

Results

Survivors with NF1 (age at diagnosis: 6.8 ± 4.8 years) had greater cumulative incidence of late mortality 30 years after diagnosis (46.3% [95% confidence interval: 23.9–62.2%]) compared with non-NF1 survivors (18.0% [16.1–20.0%]) and siblings (0.9% [0.6–1.2%]), largely due to subsequent neoplasms. Compared with survivors without NF1, those with NF1 had more severe/life-threatening chronic conditions at cohort entry (46.3% [38.1–54.4%] vs. 30.8% [29.1–32.6%]), but similar rates of new conditions during follow-up (rate ratio: 1.26 [0.90–1.77]). Survivors with NF1 were more likely to report psychosocial impairments, neurocognitive deficits, and socioeconomic difficulties compared with survivors without NF1.

Conclusions

Late mortality among glioma survivors with NF1 is twice that of other survivors, due largely to subsequent malignancies. Screening, prevention, and early intervention for chronic health conditions and psychosocial and neurocognitive deficits may reduce long-term morbidity in this vulnerable population.

中文翻译：

患有 1 型神经纤维瘤病的儿童胶质瘤成年幸存者的晚期发病率和死亡率：儿童癌症幸存者研究报告。

目的

1 型神经纤维瘤病 (NF1) 与肿瘤易感性和非恶性健康状况有关。患有 NF1 的儿童癌症幸存者是否会增加长期健康状况不佳的风险尚不清楚。

方法

将来自儿童癌症幸存者研究的 147 名 5 岁以上患有 NF1 的儿童胶质瘤幸存者与 2629 名非 NF1 胶质瘤幸存者和 5051 名兄弟姐妹的晚期死亡率、慢性健康状况以及心理社会、神经认知和社会经济结果进行了比较。

结果

与非 NF1 幸存者（18.0% [16.1 –20.0%]）和兄弟姐妹（0.9% [0.6–1.2%]），主要是由于随后的肿瘤。与没有 NF1 的幸存者相比，有 NF1 的患者在进入队列时有更严重/危及生命的慢性病（46.3% [38.1-54.4%] vs. 30.8% [29.1-32.6%]），但在随访期间新病的发生率相似向上（比率：1.26 [0.90–1.77]）。与没有 NF1 的幸存者相比，患有 NF1 的幸存者更有可能报告社会心理障碍、神经认知缺陷和社会经济困难。