Pulmonary outcomes in survivors of childhood central nervous system malignancies: A report from the childhood cancer survivor study

Abstract

Background Adult survivors of childhood Central Nervous System (CNS) tumors may be at risk for pulmonary dysfunction. This study enumerates the incidence of pulmonary dysfunction and explores associations between craniospinal irradiation (CSI) and pulmonary dysfunction among survivors of childhood CNS tumors. Methods Participants included Childhood Cancer Survivor Study (CCSS) cohort members treated for CNS malignancies when < age 21, who survived five+ years, and sibling comparisons. Medical records were abstracted and participants completed questionnaires that asked about the nature and timing of pulmonary dysfunction. Incidence rates were calculated, and Poisson regression, adjusted for chemotherapy exposures, was used to evaluate associations between CSI and pulmonary dysfunction. Results Survivor participants (N=1,653) were 54.7% male, median age at diagnosis 7.6 (range 0–21), and median time from cohort entry 18.5 (range 3.3–33.9) years. The incidence of pulmonary dysfunction (per 1,000 person years) was 9.1 (95% CI 7.8–10.6) for emphysema/obliterative bronchiolitis and >3.0 for asthma, chronic cough and need for extra oxygen. Rates of fibrosis (RR 2.0, 95% CI 1.0–3.9), chest wall abnormalities (RR 19.0, 95% CI 4.2–85.7), chronic cough (RR 1.6, 95% CI 1.2–2.1) and need for supplemental oxygen (RR 2.5, 95% CI 1.9–3.3) were higher among survivors than among siblings. Survivors treated with CSI were 10.4 (95% CI 7.6–14.4) times more likely than those not exposed to report chest wall deformity. Conclusion Adult survivors of CNS malignancy have high rates of pulmonary dysfunction 5+ years after diagnosis. Survivors treated with CSI should be monitored for pulmonary disease to permit early interventions.

Long-Term Pulmonary Function in Survivors of Childhood Cancer

“…Few studies have included age-and sex-matched noncancer controls, 9,10 and none have examined the impact of abnormal lung function on health-related quality of life The Children's Oncology Group (COG) Long-Term FollowUp (LTFU) Guidelines 1,13 recommend that survivors exposed to pulmonary-toxic therapy undergo a one-time pulmonary function testing (PFT) and symptom assessment at the time of transition into LTFU care, with subsequent testing as clinically indicated. However, a paucity of information regarding the trajectory of change in pulmonary function with time has precluded the development of guidelines regarding the frequency and duration of subsequent screenings.…”

A Review of Cardiorespiratory Fitness in Adolescent and Young Adult Survivors of Childhood Cancer: Factors that Affect its Decline and Opportunities for Intervention

“…35 Similarly, after a median of 18 years of follow-up among childhood cancer survivors treated with bleomycin, Mulder et al found pulmonary function impairments in 44% of survivors. 36 37 Finally, using a comprehensive systematic clinical assessment, Hudson et al found that the prevalence of adverse outcomes by organ system among adult survivors of childhood cancer was highest for the pulmonary system, with abnormal pulmonary function found in 65.2% of survivors after a median time from diagnosis of 25 years. 38 …”

Acute toxicity of craniospinal irradiation with volumetric‐modulated arc therapy in children with solid tumors

“…This puts long term survivors at significant risk of long term sequelae of RT as they age. The sequelae include hearing loss, cardiac disease, hypothyroidism, neurocognitive decline, vertebral body impaired growth, or secondary malignancies …”