Core deficits and quality of survival after childhood medulloblastoma: a review

Abstract

Background. Medulloblastoma is the most common malignant central nervous system tumor in children. Treatment most often includes surgical resection, craniospinal irradiation, and adjuvant chemotherapy. Although survival has improved dramatically, the tumor and its treatments have devastating long-term side effects that negatively impact quality of survival (QoS). The objective was to review the literature on QoS following childhood medulloblastoma. Methods. This narrative review is based on a Medline database search and examination of the reference lists of papers selected. Results. Frequent problems after medulloblastoma treatment include medical complications, such as long-term neurological and sensory (hearing loss) impairments; endocrine deficits, including growth problems; and secondary tumors. Neurocognitive impairment is repeatedly reported, with decreasing cognitive performances over time. Although all cognitive domains may be affected, low processing speed, attention difficulties, and working memory difficulties are described as the core cognitive deficits resulting from both cerebellar damage and the negative effect of radiation on white matter development. Long-term psychosocial limitations include low academic achievement, unemployment, and poor community integration with social isolation. Important negative prognostic factors include young age at diagnosis, conventional craniospinal radiotherapy, presence of postoperative cerebellar mutism, and perioperative complications. The influence of environmental factors, such as family background and interventions, remains understudied. Conclusion. Future studies should focus on the respective impact of radiation, cerebellar damage, genomic and molecular subgroup parameters, and environmental factors on cognitive and psychosocial outcomes. Long-term (probably lifelong) follow-up into adulthood is required in order to monitor development and implement timely, suitable, multi-disciplinary rehabilitation interventions and special education or support when necessary.

Assessment of executive functions in children and adolescents with acquired brain injury (ABI) using a novel complex multi-tasking computerised task: The Jansari assessment of Executive Functions for Children (JEF-C©)

“…Roughly 20-40% of children experiencing TBI between 5-15 years of age show significant executive dysfunction within the first year of injury (Sesma, Slomine, Ding, & McCarthy, 2008). In studies of EFs following childhood ABI, impairments have been found across the entire range of executive constructs (Beauchamp et al, 2011;Chevignard et al, 2016;Chevignard et al, 2017;Greenham et al, 2017;Krasny-Pacini et al, 2016). Specifically, EFs consisting of self-generative behavior, flexibility, set shifting, mental control, attention, and selfmonitoring are commonly impaired in pediatric populations following TBI (Baron, 2004;Chevignard et al, 2017;Gioia, Isquith, & Guy, 2001;Krasny-Pacini et al, 2016;Shultz et al, 2016) with persistent difficulties documented up to 10 years post-injury (Beauchamp et al, 2011).…”

“…Children who sustain ABI are likely to exhibit physical, neurological, cognitive, emotional, psychosocial and behavioral impairments (Greenham, Anderson, & Mackay, 2017), such as epilepsy, impaired cognition (Chevignard, Câmara-Costa, Doz, & Dellatolas, 2016) or communication (Doser, Poulsen, & Norup, 2015), post-traumatic stress disorder (PTSD) (Von Steinbuechel et al, 2016), or depression (Letkiewicz et al, 2014). They are also likely to be bullied, to bully others, and to display violent as well as non-violent conduct behaviors (Ilie et al, 2014).…”

“…They are also likely to be bullied, to bully others, and to display violent as well as non-violent conduct behaviors (Ilie et al, 2014). Long-term psychosocial limitations include low academic achievement, unemployment, and poor community integration with social isolation (Chevignard et al, 2016).…”

Quality of survival and cognitive performance in children treated for medulloblastoma in the PNET 4 randomized controlled trial

“…post-operative cerebellar mutism, ataxia) and treatment-related factors (e.g. radiation dose and volumes) 30 . Thus, the influence of these factors should be considered when examining QoS.…”

“…Similarly, in the present study, the association of these complications was moderate with IQ measures, whereas it was non-significant with the questionnaire-based measures. However, a negative influence of post-operative complications on QoS assessed through questionnaires has been reported, although their influence on psychosocial domains seems to be less pronounced and has been less frequently studied 30 .…”