ABSTRACT
PURPOSE: To analyze the relationship between craniospinal irradiation (CSI) and intellectual outcome in children with posterior fossa (PF) tumors. METHODS AND MATERIALS: A neuropsychological evaluation was performed retrospectively in 31 children, aged 5-15 years, who had received radiotherapy for PF tumors, and who had been off therapy for at least 1 year. Factors evaluated for impact on intellectual outcome were: socioeconomic status, disease presentation, histology, complications, chemotherapy, age at radiotherapy, interval between radiotherapy and testing, and radiation doses and volumes. Patients were divided into 3 subgroups according to the CSI doses (0 Gy [i.e., PF irradiation only], 25 Gy, and 35 Gy), with 11, 11, and 9 patients, respectively. RESULTS: Long-term cognitive impairment occurred in most of the patients, even after PF irradiation only. Moreover, there was a significant correlation between the full-scale IQ score (FSIQ) and the CSI dose, with mean FSIQ scores at 84.5 (SD = 14.0), 76.9 (SD = 16.6), and 63.7 (SD = 15.4) for 0 Gy, 25 Gy, and 35 Gy of CS1, respectively. A marked drop in verbal comprehension scores was noted in children who had received the higher dose. CONCLUSION: This preliminary study further supports the rationale for de-escalation of CSI doses and volumes in standard-risk PF tumors.
Subject(s)
Cerebellar Neoplasms/radiotherapy , Ependymoma/radiotherapy , Intelligence/radiation effects , Medulloblastoma/radiotherapy , Adolescent , Cerebellar Neoplasms/physiopathology , Child , Child, Preschool , Cognition/radiation effects , Cranial Fossa, Posterior , Educational Status , Ependymoma/physiopathology , Female , Follow-Up Studies , Humans , Male , Medulloblastoma/physiopathology , Neuropsychological Tests , Radiotherapy Dosage , Retrospective StudiesABSTRACT
We present the clinical observation of a 16-month-old girl treated for a posterior fossa ependymoma who experienced severe and delayed visual dysfunction. She was initially treated by surgery and conventional chemotherapy. When she relapsed at age 3 years, the salvage treatment combined high-dose chemotherapy, second surgery, and local irradiation. At age 4 years, disturbed gait and dysarthric speech appeared rapidly, and she became unable to recognize objects and people. Computed tomography revealed bilateral calcifications in the cerebellum and temporal and occipital lobes but no relapse. The neuropsychologic evaluations revealed signs of visual agnosia and marked intellectual impairment. The role of the different treatment modalities in the pathogenesis of this unusual syndrome is discussed.