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Abstract
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.
Details
; Li, Nan 2 ; Fisher, Michael J 3 ; Ullrich, Nicole J 4 ; Bhatia Smita 5 ; Yasui Yutaka 2 ; Sklar, Charles A 6 ; Leisenring, Wendy 7 ; Howell, Rebecca 8 ; Oeffinger, Kevin 9 ; Hardy, Kristina 10 ; Fatih, Okcu M 11 ; Gibson, Todd M 2 ; Robison, Leslie L 2 ; Armstrong, Gregory T 2 ; Krull, Kevin R 2 1 University of Cincinnati and Cincinnati Children’s Hospital Medical Center, Cincinnati, USA (GRID:grid.239573.9) (ISNI:0000 0000 9025 8099)
2 St. Jude Children’s Research Hospital, Departments of Epidemiology and Cancer Control, Oncology and Psychology, Memphis, USA (GRID:grid.240871.8) (ISNI:0000 0001 0224 711X)
3 University of Pennsylvania Perlman School of Medicine, Children’s Hospital of Philadelphia, Philadelphia, USA (GRID:grid.239552.a) (ISNI:0000 0001 0680 8770)
4 Harvard Medical School, Boston Children’s Hospital, Boston, USA (GRID:grid.239552.a)
5 University of Alabama at Birmingham, Institute for Cancer Outcomes and Survivorship, Birmingham, USA (GRID:grid.265892.2) (ISNI:0000000106344187)
6 Memorial Sloan Kettering Cancer Center, New York, USA (GRID:grid.51462.34) (ISNI:0000 0001 2171 9952)
7 Fred Hutchinson Cancer Research Center, Cancer Prevention and Clinical Statistics Programs, Seattle, USA (GRID:grid.270240.3) (ISNI:0000 0001 2180 1622)
8 The University of Texas MD Anderson Cancer Center, Department of Radiation Physics, Houston, USA (GRID:grid.240145.6) (ISNI:0000 0001 2291 4776)
9 Duke University, Department of Medicine, Durham, USA (GRID:grid.26009.3d) (ISNI:0000 0004 1936 7961)
10 Children’s National Medical Center, Washington, USA (GRID:grid.239560.b) (ISNI:0000 0004 0482 1586)
11 Baylor College of Medicine, Texas Children’s Hospital, Houston, USA (GRID:grid.416975.8) (ISNI:0000 0001 2200 2638)