Neuropsychological resiliency after treatment for advanced stage neuroblastoma.

The purpose of this study was to describe the neuropsychological functioning of survivors of advanced stage neuroblastoma. In all, 16 survivors, diagnosed at a median of 2.8 years, who had received intensive chemotherapy and surgical treatments, were identified; 11 had received myeloablative consolidation therapy, eight with total body irradiation (TBI). All patients were evaluated with a neuropsychological assessment battery at a median age of 8.8 years. Analyses included comparison of the performances of the TBI group vs the no-TBI group; determination of whether the proportion of individuals with impaired or superior performance on each measure exceeded normative expectations; and performance indexes reflecting patterns of performance. Results indicate no significant deleterious impact of TBI and/or presence or absence of myeloablative therapy on neurocognitive and neurobehavioral functioning. For this cohort, resilience to neuropsychological vulnerability was observed, which included the emergence of a profile of full-scale IQ, verbal IQ, and mathematical achievement well above average expectations. We concluded that the results document a lack of neuropsychological morbidity among this cohort of survivors of advanced stage neuroblastoma, regardless of the inclusion of TBI. Moreover, a striking pattern of excellent neurocognitive functioning with intact neurobehavioral functioning was observed.

Excellent therapeutic efficacy and minimal late neurotoxicity in children treated with 18 grays of cranial radiation therapy for high-risk acute lymphoblastic leukemia: a 7-year follow-up study of the Dana-Farber Cancer Institute Consortium Protocol 87-01.

BACKGROUND: In the current study, the authors evaluated late neuropsychologic effects 7 years after diagnosis and the long-term survival in a cohort of patients treated for high-risk childhood acute lymphoblastic leukemia (ALL) with cranial radiation therapy. Efficacy and toxicity were evaluated in relation to patient age at diagnosis (age < or > or = 36 months). METHODS: Two hundred and one patients treated for high-risk ALL on the Dana-Farber Cancer Institute Consortium Protocol 87-01 were included, 147 of whom were in continuous complete disease remission and were eligible for cognitive testing. Sixty-one patients consented to undergo testing. All patients received 18 grays (Gy) of cranial radiation as a component of central nervous system treatment. RESULTS: For all 201 patients, the 5-year overall survival (% +/- the standard error) was 82% +/- 2 and the 5-year event-free survival (% +/- the standard error) was 75% +/- 3. Only two patients developed a central nervous system recurrence. Intelligence quotient (IQ) and memory were at the expected mean for age, but performance on a complex figure drawing task was found to be reduced. Children who were age < 36 months at the time of diagnosis were found to have an IQ in the average range, but showed verbal deficits. CONCLUSIONS: The results of the current study demonstrate excellent efficacy of therapy and relatively limited late neurotoxicity on a childhood ALL therapy protocol in which all evaluated patients had received 18 Gy of cranial radiation. Efficacious therapy that includes cranial radiation does not appear to necessarily incur a heightened risk for significant cognitive impairment.

Memory deficits among children with craniopharyngiomas.

OBJECTIVE: To describe neuropsychological functioning (with a specific focus on cognition and memory) after surgical treatment of craniopharyngiomas. METHODS: Sixteen patients who were between 6 and 15 years of age at the time of surgery comprised the sample. Each child had been treated for a craniopharyngioma with surgery only, on Dana-Farber Cancer Institute Protocol 92-077. RESULTS: The overall level of cognitive functioning was well within the average range, with both language and visuospatial functioning being generally intact; however, specific memory problems, in both the language and visuospatial domains, were evident. CONCLUSION: Although general cognitive functioning was intact after the surgical treatment of craniopharyngiomas, difficulties in the retrieval of learned information were observed. Neuropsychological assessments, with a focus on memory recall, should be a component of the medical management plan for each child.

Cognitive sequelae in children treated for acute lymphoblastic leukemia with dexamethasone or prednisone.

PURPOSE: The cognitive sequelae of treatment for childhood acute lymphoblastic leukemia (ALL) were compared in a group of patients who received dexamethasone during the intensification and maintenance phases of therapy with those in a historical control group for whom antileukemia therapy was similar, except that the corticosteroid component of therapy was prednisone. METHODS: Patients treated for ALL on Dana-Farber Cancer Institute protocols 87-01 (n = 44) and 91-01 (n = 23) were evaluated by standard cognitive and achievement tests. Corticosteroid therapy was delivered in 5-day pulses given every 3 weeks during intensification and continuation phases of therapy for a total of 2 years. RESULTS: Children treated on protocol 87-01 received prednisone at a dose of 40 mg/m2/d (standard risk, SR) or 120 mg/ m2/d (high risk, HR); those treated on protocol 91-01 received dexamethasone at a dose of 6 mg/m2 per day (SR) or 18 mg/m2 per day (HR). Children treated on protocol 91-01 performed less well on cognitive testing. Subsample analysis indicated that cranial radiation therapy and methotrexate dose did not account for differences in cognitive outcomes. CONCLUSIONS: The findings of this preliminary study are consistent with the hypothesis that dexamethasone therapy can increase risk for neurocognitive late effects in children treated for ALL and indicate that further investigation of this question is warranted.

Patterns of memory dysfunction among children surviving temporal lobe tumors.

We compared the cognitive functioning of seven children with left and seven children with right temporal lobe tumors one year or more following completion of medical intervention (surgical resection + radiation therapy). No differences were found between the two groups with respect to verbal IQ, performance IQ.full scale IQ, verbal minus performance IQ, or verbal comprehension, perceptual organization, or freedom from distractability factor scores. After adjusting memory test results for variance attributable to general intelligence, three children with left and four children with right temporal tumors exhibited specific auditory-verbal memory dysfunction; five of these children had concurrent visual-verbal memory dysfunction. Children receiving radiation therapy to the tumor were at significantly greater risk for verbal memory problems. Reading and spelling achievement were significantly correlated with verbal memory performance. Inconsistent association between cerebral hemisphere of tumor and type of cognitive deficit may be related to the relatively slow progression of the tumor and regional interventions such as radiation therapy. Nevertheless, the results of the present report suggest that the majority of children diagnosed with temporal lobe tumors are at increased risk for memory dysfunction and academic failure.