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NFM-03. EFFECT OF NEUROFIBROMATOSIS TYPE 1 ON LONG-TERM HEALTH OUTCOMES IN ADULT SURVIVORS OF CHILDHOOD ASTROCYTOMA: A REPORT FROM THE CHILDHOOD CANCER SURVIVOR STUDY
Abstract
PURPOSE
The effect of neurofibromatosis type 1 (NF1) on incidence and severity of health outcomes in adult survivors of childhood astrocytoma is poorly understood.
METHODS
1,617 >5yr survivors of childhood astrocytoma (147 NF1 survivors and 1,470 non-NF1 survivors matched on diagnosis and diagnosis decade) and 5,051 siblings were compared on self-reported chronic medical conditions (CTCAE v4.0) and cognitive impairment (defined as >90 th percentile of siblings on the CCSS Neurocognitive Questionnaire) using logistic and Poisson regression adjusted for age at survey, age at diagnosis, sex, race, CNS radiation and other treatment exposures.
RESULTS
Among astrocytoma survivors, NF1 survivors (mean age 6.6yr at diagnosis) received chemotherapy (24% vs 13%) and carboplatin (71% vs 40%) more frequently than non-NF1 survivors. Compared to non-NF1 survivors, NF1 survivors were more likely to report a serious health condition (grade 3-4, RR[95%CI] 1.9[1.5-2.4]) and multiple chronic medical conditions (grade 2-4, 1.7[1.4-2.1]). Specifically, NF1 survivors were at increased risk for vision loss (1.8[1.5-2.1]), gastrointestinal disease (1.8[1.5-2.1]), heart disease (1.5[1.2-2.0]), lung disease (1.4[1.2-1.6]), and motor (1.2[1.1-1.4]) and sensory (1.2[1.0-1.4]) impairments. Interestingly, compared to both non-NF1 survivors and siblings, diabetes (0.2[0.1-0.3] vs non-NF1; 0.3[0.2-0.5] vs siblings) and abnormal thyroid (0.2[0.2-0.3] vs non-NF1; 0.6[0.5-0.8] vs siblings) were less common in NF1 survivors. NF1 survivors reported more impairment on emotional regulation (OR 2.0[1.1-3.7]), task completion (2.0[1.2-3.5]) and learning/memory (1.5[1.3-1.8]), and attended college less frequently (0.5[0.2-0.9]) compared to non-NF1 survivors.