Prognostic signs in the surgical management of plexiform neurofibroma: the Children's Hospital of Philadelphia experience, 1974-1994.

OBJECTIVES: To estimate the rate of progression of plexiform neurofibroma after surgery and to identify prognostic factors that predict progression. STUDY DESIGN: A retrospective review of the inpatient and outpatient records of 121 patients, who had 302 procedures on 168 tumors over a 20-year period at a single large pediatric referral center. Data on age, location, indication for surgery, and extent of resection was analyzed for prognostic significance. RESULTS: The overall freedom from progression was 54%. Children < 10 years old had a shorter interval of tumor control than older children (p = 0.0004). Tumors of the head/neck/face fared worse than tumors of the extremities (p = 0.0003). Less extensive resection predicted shorter interval to progression (p < 0.0001). Indication for surgery was not of prognostic importance. In multivariable analysis older age and location in the extremities were predictors of a better outcome. CONCLUSIONS: Tumor progression is a serious problem for children with plexiform neurofibroma. Younger children, children with tumors of the head/neck/face, and tumors that cannot be nearly completely removed are at particular risk. These data may be useful in helping clinicians decide which patients and which tumors are most likely to benefit from surgical intervention.

Cognitive deficits in long-term survivors of childhood medulloblastoma and other noncortical tumors: age-dependent effects of whole brain radiation.

Twenty-four children, aged 1.5-20 yr at diagnosis, with noncortical brain tumors, primarily medulloblastoma, have been followed for 3-4 yr for intellectual status. All the children received craniospinal irradiation, and 19 of 24 received chemotherapy as well. For the group as a whole. Full Scale IQ fell from 104 at baseline to 91 at final follow-up. Children younger than 7 yr at diagnosis showed a significant decrease in IQ as early as year 1, and all changes from baseline to years 3 and 4 were significant. In contrast, children older than 7 yr at diagnosis did not show a significant IQ change from baseline to year 3 or 4. The Spearman correlation coefficient between IQ change and age at diagnosis from baseline to year 4 was 0.57 (P = 0.003). This study supports the hypothesis that children treated with whole brain radiation at a younger age have more severe cognitive impairment than those treated at a later age. Limitations in sample size and duration of observations do not permit us to identify whether a true plateau occurs 2-4 yr after irradiation versus a continued progressive decline in intellectual performance. Moreover, we cannot at this time distinguish between a true dementing process versus failure to acquire new cognitive skills at a rate comparable to age-matched peers.