Impact of radiation avoidance on survival and neurocognitive outcome in infant medulloblastoma

[摘要] Purpose: Concerns regarding radiotherapy-related neurocognitive sequelae in young children have led to deferral and/or avoidance of radiation in contemporary treatment for this fragile group of patients. We sought to compare survival and neurocognitive outcome of two groups of infants with medulloblastoma who received adjuvant conventional craniospinal irradiation (CSI), versus reduced or no radiotherapy during an era of change in infant medulloblastoma treatment philosophy.Patients and Methods: From 1985 to 2007, 29 patients3 years of age were diagnosed and treated with curative intent in our institution. Children treated prior to 1994 received adjuvant radiation with chemotherapy; subsequently, radiation was essentially prescribed for disease progression or relapse.Results: Median age at diagnosis was 24 months (range 1-36); 15 patients (52%) presented with metastatic disease at diagnosis. Eight children received adjuvant radiotherapy with chemotherapy and 21 children received post-operative chemotherapy only as part of their initial treatment. Five children treated with chemotherapy alone are in prolonged remission. Five years EFS and OS were 35.9% (+/-9.8%) and 50.2% (+/- 9.6%) respectively. Extent of resection, metastatic status and desmoplastic histology were not found to be significant prognostic factors. On serial neurocognitive evaluations, patients treated with chemotherapy +/- reduced radiotherapy demonstrated improvement of intellectual function overtime. Patients treated with conventional craniospinal irradiation (CSI) exhibited significantly lower IQ and academic performances except for receptive vocabulary. Conclusion:Avoidance of conventional CSI in treatment of very young children with medulloblastoma appears to be associated with preserved neurocognitive profile. Neurocognitive evaluation should be integrated in the primary objectives of future infant protocols.

[学科分类] 肿瘤学