Long-term neurologic health and psychosocial function of adult survivors of childhood medulloblastoma/PNET: a report from the Childhood Cancer Survivor Study

Long-term neurologic health and psychosocial function of adult survivors of childhood medulloblastoma/PNET: a report from the Childhood Cancer Survivor Study
The authors wanted to investigate the long term neurologic and psychosocial outcomes for adult survivors of pediatric medulloblastoma/PNET who were treated with craniospinal irradiation (CSI) and chemotherapy. This is a retrospective cohort study.
The authors used data from the Childhood Cancer Survival Study (CCSS) with patients diagnosed between 1970-1986. They randomly selected a subset of survivors of medulloblastoma/PNET, and identified and recruited siblings closest in age. A self-administered baseline questionnaire was given to survivors and siblings to understand demographics and health-outcomes. Cancer diagnosis and treatment details including chemotherapy protocol and radiation exposure were abstracted from the medical chart using CCSS protocol.
The authors recruited 380 survivors and 4031 sibling pairs to the study. 73% of suvivors were 5 years from diagnosis. Forty patients had a stroke, with 25 reported >5 years from diagnosis. Of notes, none of the cumulative incidence curves leveled off at 30 years, indicating patients were still suffering events for decades after diagnosis. 70.6% of patients were alive 30 years from diagnosis. Primary tumor recurrence was 18%. 30-year incidence of 2nd malignant neoplasm was 8% (thyroid cancer, malignant brain tumor, sarcoma). Only 25% of survivors achieved a bachelor's degree.
Retrospective study, it only represents about 30% of patients diagnosed from 1970-1986, so there could be some bias. There is also no separation between those with PNET and medulloblastoma, although they were all treated similarly in this era. This is also not representative of what many patients would be treated with today (reduced CSI dose for SR medulloblastoma of 23.4 Gy, all patients would typically receive chemotherapy), so it may not be generalizable to patients undergoing treatment today.
Survivors of medulloblastoma/PNET are at increased risk for neurologic deficits, lower academic achievement and underemployment as adults. Neurologic events, including hearing loss, seizures, cataract development and stroke continued to occur up to 30 years from diagnosis. Long term follow up including regular hearing and vision assessment is important in this population.