Kim Bull

Reduction of Health Status 7 Years After Addition of Chemotherapy to Craniospinal Irradiation for Medulloblastoma: A Follow-Up Study in PNET 3 Trial Survivors—on Behalf of the CCLG (formerly UKCCSG)

PURPOSE To compare quality of survival after craniospinal irradiation (CSI) alone with survival after CSI plus chemotherapy (CT) for medulloblastoma. PATIENTS AND METHODS Follow-up study of surviving UK patients with medulloblastoma diagnosed between 1992 and 2000 treated according to one or other treatment arm of the PNET 3 controlled trial. RESULTS Seventy three percent of all 147 eligible patients ages 6.6 to 24.3 years were assessed at a mean of 7.2 years after diagnosis. Health status was significantly poorer in the group treated in the CSI plus CT arm of the trial than in the CSI alone arm, and there were also trends to poorer outcomes for behavior and quality of life scores. The CSI plus CT group were also significantly more restricted physically and needed more therapeutic and educational support. Body mass index, stature, and other endocrine outcomes were similar in the two treatment arms, except for the trend in increased frequency of medical induction of puberty in the CSI plus CT group. CONCLUSION The addition of CT to CSI for medulloblastoma was associated with a significant decrease in health status. The effect of the addition of other CT regimens to CSI on quality of survival should be evaluated.

Screening for cognitive deficits in 8 to 14-year old children with cerebellar tumors using self-report measures of executive and behavioral functioning and health-related quality of life

BACKGROUND We aimed to identify a brief screening measure for detection of cognitive deficit in children treated for cerebellar tumors that would be useful in clinical practice. METHODS A sample of 72 children, aged 8-14 years, and within 3 years post diagnosis for standard-risk medulloblastoma (n = 37) or low-grade cerebellar astrocytoma (n = 35) and 38 children in a nontumor group were assessed using teacher-, parent-, and child-report of the Behavior Rating Inventory of Executive Function (BRIEF), Strengths and Difficulties Questionnaire (SDQ), and Pediatric Quality of Life Inventory (PedsQL). The accuracy of these scores as a screen for a full-scale Intelligence Quotient (FSIQ) < 80 on the Wechsler Intelligence Scale for Children (WISC-IV UK) was assessed using their receiver operating characteristic (ROC) curves. RESULTS The questionnaires with the highest areas under the ROC curves were the child- and parent-report PedsQL, the teacher-report BRIEF, and the SDQ. At optimal cutoff scores, their sensitivities (95% CIs) to cases of FSIQ < 80 were 84 (60-96)%, 65 (41-84)%, 79 (54-93)%, and 84 (60-96)%, and their specificities (95% CIs) were 79 (68-86)%, 87 (77-93)%, 77 (66-86)%, and 71 (64-84)% respectively. All cases of FSIQ < 80 screened positive on either teacher-report SDQ or self-report PedsQL. CONCLUSIONS The PedsQL child- and parent-report and the teacher-report BRIEF and SDQ have moderately good accuracy for discriminating between children with and without a FSIQ < 80. The PedsQL could be used in a clinical setting, and the BRIEF and SDQ in an educational setting, to screen for cases with FSIQ < 80 in children treated for brain tumors.

Improved health-related quality of life outcomes associated with SHH subgroup medulloblastoma in SIOP-UKCCSG PNET3 trial survivors

[Preview] Reduced health-related quality of life (HrQoL), including impaired cognitive, social, physical, behavioural and emotional functioning, is common in children treated for medulloblastoma and has been associated with clinical factors such as surgical resection and treatment modality]. recent biological advances have allowed the distinction of medulloblastomas into four consensus molecular subgroups—WNT, sHH, Group 3 and Group 4—which display distinct molecular, clinical, and pathological disease characteristics. Together, these observations raise the hypothesis that HrQoL in medulloblastoma survivors may be related to their underlying tumour biology.

Neurocognitive effects of CNS tumors

There is ample evidence that many children treated for brain tumors experience long-term neurocognitive deficits. The severity of those deficits is determined by a complex interaction of the childs genetic make-up and age, neuroanatomical damage caused by tumor and surgery, radiotherapy and chemotherapy, the psychosocial environment, and the intensity of targeted rehabilitation. The consequences of neurocognitive deficits are moderated by the number and severity of other deficits, including neurological and endocrine impairments, and this wider context must be considered. The impact of intellectual decline on academic functioning is evident, and underlies, for example, poor reading, writing, and mathematical skills. The effects of early brain damage on development are cumulative as more functions are expected to mature. Many survivors of CNS tumors can be expected to grow into deficits that have far-reaching consequences not only for academic achievement but also for their psychological and social development and their ability to be self-sufficient. Because the problems typically only become apparent over time, surveillance for their detection is an essential prerequisite for early educational and other interventions to support learning and successful transition to independent adult life.