Rene Y. McNall-Knapp

The goal of this study was to evaluate extent of surgical resection, and timing and volume of re-irradiation, on survival for children with locally recurrent ependymoma. Children with locally recurrent ependymoma treated with a second course of fractionated radiotherapy (RT2) from 6 North American cancer centers were reviewed. The index time was from the start of RT2 unless otherwise stated. Thirty-five patients were included in the study. The median doses for first radiation (RT1) and RT2 were 55.8 and 54 Gy, respectively. Median follow-up time was 5.6 years. Median overall survival (OS) for all patients from RT2 was 65 months. Gross total resection (GTR) was performed in 46% and 66% of patients prior to RT1 and RT2, respectively. GTR prior to RT2 was independently associated with improved progression-free survival (PFS) for all patients (HR 0.41, P = 0.04), with an OS benefit (HR 0.26, P = 0.03) for infratentorial tumors. Median PFS was superior with craniospinal irradiation (CSI) RT2 (not reached) compared to focal RT2 (56.9 months; log-rank P = 0.03). All distant failures (except one) occurred after focal RT2. Local failures after focal RT2 were predominantly in patients with less than GTR pre-RT2. Patients with locally recurrent pediatric ependymoma should be considered for re-treatment with repeat maximal safe resection (ideally GTR) and CSI re-irradiation, with careful discussion of the potential side effects of these treatments.

Objective: Symptom burden of children with cancer appears to contribute to parent distress, but the mechanisms of this relationship are relatively unexplored. The current cross-sectional study examined rumination (i.e., repeated focus on negative events and experiences) as a mediator of the associations between parent-report of child (a) pain or (b) nausea, and parent posttraumatic stress symptoms (PTSS); parent-report of child symptoms → parent rumination → parent PTSS. Methods: Caregivers (N = 40, M _Age = 38.7 years, female = 87.5%, White = 67.5%) of children with cancer ( M _Age = 9.4 years) were recruited at a Midwest pediatric cancer center following their child’s diagnosis ( M _Months = 2.97 ± 1.98). Parents completed the PedsQL-Cancer Module, the Ruminative Response Scale, and the Impact of Events Scale—Revised. To test the hypothesized indirect effects, 2 separate regression models specifying 5,000 bias-corrected bootstrapping resamples were conducted via Hayes’ PROCESS macro. Family income served as a covariate. Results: Bootstrap regression analysis revealed that parent-report of child pain had an indirect effect on parent PTSS via parent rumination, controlling for family income, R2 = .37, ß = −.24, 95% CI [−.494, −.033]. Child nausea demonstrated an indirect effect on parent PTSS via parent rumination, controlling for family income, R2 = .36, ß = −.22, 95% CI [−.428, −.008]. Conclusions: Findings indicate that worse parent perception of child symptoms may lead to elevated parent distress through increased rumination. These results support the role of pediatric psychologists and the interdisciplinary team in alleviating parent distress through clinical management of both child physical symptoms and parent rumination. Implications for Impact Statement: Parents of children with cancer may feel greater psychological distress when they see their child feeling physically ill (e.g., experiencing pain, experiencing nausea). This study indicates that 1 particular parent coping strategy, rumination (i.e., intensive negative thinking about their child’s illness), may explain how parental perceptions of pain or nausea lead to greater parental distress. Addressing child symptoms as part of palliative care is essential for the well-being of the whole family, and parents may benefit from increased involvement in the clinical symptom management process and from learning adaptive strategies for coping with cancer-related distress.

Acute lymphoblastic leukemia (ALL) is the most common childhood cancer.¹ Although more than 95% of children with ALL enter remission after a 4-week induction, approximately 20% relapse within 5 years.¹ Second-line therapies are toxic, and salvage is poor.² Durable first clinical remission is desirable and requires a prolonged maintenance phase with daily self-administered oral mercaptopurine. Low mercaptopurine systemic exposure increases relapse risk.^3-5 Patient adherence to mercaptopurine is a primary determinant of mercaptopurine systemic exposure.⁶ Adherence to medication is a process by which patients take their medication as prescribed and typically consists of 3 phases: initiation, implementation, and discontinuation.^7,8 In the present study and our prior studies,⁸ we focus on the implementation phase of mercaptopurine adherence, in which patients have initiated but have not yet discontinued mercaptopurine. In a previous Children’s Oncology Group (COG) study (AALL03N1), we measured mercaptopurine adherence electronically in children with ALL entering maintenance during the first clinical remission, using the Medication Event Monitoring System (MEMS) TrackCap device (Aprex Corp).^6,9,10 Poor mercaptopurine adherence (mean adherence rates, 6^,9,10 The most common reason for missing mercaptopurine was forgetfulness⁹; adherent patients and parents endorsed parental vigilance as a strategy to overcome forgetfulness.¹¹ Given the critical need to improve mercaptopurine adherence in children with ALL, we conducted a randomized clinical trial (COG ACCL1033) in which children with ALL receiving mercaptopurine maintenance were randomly assigned to receive either education alone or a comprehensive adherence-enhancing intervention in addition to education. We wished to test the hypothesis that the adherence-enhancing intervention consisting of education and daily text message reminders to prompt directly supervised therapy (DST) would result in a higher proportion of patients with mercaptopurine adherence rates 95% or higher compared with education alone.

Purpose: To report on a novel translocation related to a suprasellar primitive neuroectodermal tumor (sPNET) and retinoblastoma. Design: Case report. Methods: A 6-year-old girl underwent genetic testing after developing unilateral retinoblastoma subsequent to treatment (surgery, chemotherapy, and stem-cell rescue) for a sPNET found at 1 year of age. Results: Genetic testing found the girl’s karyotype to be 46,XX,t(11;13)(q21;q14.2); a novel translocation not previously reported in patients with either retinoblastoma or sPNET. Conclusions: Our patient had a novel translocation affecting the retinoblastoma 1 (RB1) gene, 46,XX,t(11;13)(q21;q14.2) resulting in the late development of unilateral retinoblastoma. Although she only developed unilateral retinoblastoma, her central nervous system was affected at a very early age. How her complex mutation resulted in retinoblastoma and antecedent sPNET remains unknown.