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

Abstract

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. This narrative review is based on a Medline database search and examination of the reference lists of papers selected. 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. 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.