Abstract
Background: Tumors in the central nervous system (CNS) are the leading cause of cancer-related child mortality, with medulloblastoma (MB) and supratentorial primitive neuroectodermal tumor (CNS-PNET) among the most common. Objective: The purpose of this study was two-fold: 1) to give a descriptive characterization of survivors of MB and CNS-PNET regarding cognitive function, vision, hearing, fine motor skills, interpersonal function, and emotional distress, and 2) to understand the major factors in determining this heterogeneity, by evaluating the importance of various parameters on psychological functional outcome within this patient group. Method: Forty-two survivors of pediatric MB and CNS-PNET underwent cognitive assessment, extensive somatic evaluation, and completed a questionnaire on quality of life (QoL). The Transactional Stress and Coping Model (TSCM) was adapted to fit our patient population. Results: Many survivors experienced significant morbidity, both somatically and psychologically, but outcome was heterogeneous. Late effects ranged from adverse difficulties to none, in regards to cognitive function, motor skills, vision, hearing, interpersonal functioning, endocrine and neurological function ranging from adverse difficulties to no impairment. Our revised TSCM explained substantial variance in QoL, with an η2 = 0.57 and a more conservative ⍵^2 = 0.36. Interpersonal functioning was significantly influenced by severity and type of endocrine illness and by coping ability, as measured by working memory and cognitive control. Emotional distress was significantly influenced by endocrine illness and neurological illness. Conclusion: This study finds that survivors of MB and CNS-PNET have a range of sequelae. The factors determining the variations in well-being included both participants' physical stressors in terms of illness severity and the patients' coping ability in terms of working memory and cognitive control. These findings may guide the long-term follow-up of these patients, e.g., neurocognitive interventions to mitigate the impact of cognitive late effects and psychotherapeutic interventions that address relational functioning.