From childhood to adulthood: long-term outcome of medulloblastoma patients. The Institut Curie experience (1980–2000)

Abstract

Medulloblastoma patients treated at the Institute Curie between 1980 and 2000 were reviewed. Only patients whose primary treatment included craniospinal radiation were considered. Surviving patients were identified and evaluated by means of self-report questionnaires using the Health Utility Index (HUI). Psychosocial functioning, employment, and other health-related indicators were recorded. Seventy-three patients were treated during the study period. At a median follow-up from diagnosis of 14.4 years, 49 patients were alive and 45 surviving patients could be contacted. Late sequelae were frequent, particularly neurological deficits (71%) and endocrine complications (52%). Impairments of psychosocial functioning, including employment, driving capacity, independent living, and marital status, were identified in most patients. Most long-term medulloblastoma survivors suffer persistent deficits in several domains, with a significant impact on their psychosocial functioning. These findings reinforce the importance of early intervention programs for all survivors in order to reduce the psychosocial impacts of their disease.

Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs)

“…1,2 In those who survive this disease, the tumor and its treatment continue to cause effects into adulthood: 55% of survivors require endocrine-replacement therapy, and 78% have residual neurologic deficits post-treatment. 3 Recently reported overall survival rates for both children and adults are in the 60th and 70th percentiles, a significant increase from the 29% 5-year overall survival rate reported in earlier periods. 2,[4][5][6][7][8][9][10] Studies have demonstrated that medulloblastomas in adults and children are histologically and genetically different diseases.…”

Selective BCL-XL inhibition promotes apoptosis in combination with MLN8237 in medulloblastoma and pediatric glioblastoma cells

“…4 However, effective treatment options for high-risk patients with poor prognosis tumors are limited, and current therapies lack target specificity and are associated with significant neurocognitive sequelae and psychosocial deficits. 5,6 Tumor recurrence following conventional treatment remains a significant problem and the majority of patients with relapsed medulloblastoma succumb to the disease. 7 Other high grade tumors including glioblastoma, are rarer in children and adolescents (<3.0% of CNS tumors).…”

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

“…Medulloblastoma (MB), the most common primary malignant brain tumor of the central nervous system (CNS) during childhood 1-3 , carries long-term implications for patients' survivorship, such as neurological and cognitive deficits 4,5 , auditory and endocrine impairments 6,7 , and the perception of reduced healthrelated quality of life (HRQoL) 8 . Increased survival rates of patients with MB have led to the recognition of the importance of comprehensive assessments aimed at providing a more complete description of survivors' quality of survival (QoS) across several domains of functioning including an individual's perception of his or her cognitive performance, health status, behavior, and HRQoL.…”