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Children with cancer experience suffering, particularly at the end of life. Pediatric hematology/oncology (PHO) fellows need dedicated palliative care (PC) training in order to adequately manage this suffering. Our objectives were to understand (1) the PC training needs of Canadian PHO fellows and (2) experiences in providing PC, from the perspectives of fellows and their training program directors (PDs) and to describe (1) our experience in enhancing our institutional PC curriculum and (2) the preliminary evaluation of this curriculum. Electronic surveys were sent to all Canadian PHO fellows and PDs. Fellows participating in our curriculum were also sent post-course surveys. All 9/9 of the PDs and 63% (29/46) of the fellows completed our pre-course surveys. The majority of survey participants agreed that PHO fellows require dedicated PC training. All programs provided some PC education, but 45% of programs offered 3 or fewer hours of training per year. Only 55% (5/9) of the PDs believed that their trainees had adequate PC skills on completion of training. Fellows perceived a range of PC skills to be important but expressed low levels of comfort across these skills. Many fellows had experienced distress as a result of managing PC clinical situations, and many cited a lack of training as contributing to their distress. Despite increasing awareness of the importance of PC education for PHO fellows, this subject does not receive adequate attention in training curricula. The introduction of a Canadian national curriculum may improve the provision of PC training in education programs.

Background Due to the long-term sequelae of cranial radiotherapy (CRT), contemporary treatment protocols for children with acute lymphoblastic leukemia (ALL) aim to limit the use of prophylactic CRT. For patients with central nervous system (CNS) involvement with ALL at diagnosis, the use of CRT remains common. Children

Purpose of review To provide an updated overview of the pathogenesis and treatment of Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD). Recent findings There is ongoing debate as to the exact pathogenesis of these disorders and their classification as reactive versus neoplastic. Proinflammatory cytokines are known to play a role in both LCH and ECD and strengthen the hypothesis that, at least in part, they are disorders of immune dysregulation. The recent discovery of activating mutations in the proto-oncogene BRAF in a subset of LCH patients suggests that LCH is in fact a neoplastic disorder. Understanding of the mechanisms that promote proliferation and migration of histiocytes has led researchers to explore targeted immune-modulatory therapies for ECD. Similarly for LCH, alternative chemotherapeutic agents and reduced-intensity hematopoietic stem cell transplant are being evaluated for refractory disease. Summary More research is needed to better understand the cause of these disorders and may help in identifying new targeted therapies, particularly for patients with refractory or relapsed disease. Multinational trials are ongoing for LCH and are urgently needed for ECD.

Due to the long-term sequelae of cranial radiotherapy (CRT), contemporary treatment protocols for children with acute lymphoblastic leukemia (ALL) aim to limit the use of prophylactic CRT. For patients with central nervous system (CNS) involvement with ALL at diagnosis, the use of CRT remains common. Children5 years of age are a particularly challenging subgroup in whom the consequences of CRT can be devastating.This study retrospectively describes the overall (OS) and event-free survival (EFS) of young children (1-5 years) who were treated for CNS-positive ALL at the Hospital for Sick Children between 2000 and 2013.Of a total of 19 patients, two were treated with upfront CRT, both as part of the conditioning regimen prior to HSCT. All patients received intensification of CNS-directed chemotherapy by triple intra-thecal chemotherapy (84.2%), use of dexamethasone in induction (57.9%) and maintenance (66.7%), and high-dose methotrexate (77.8%). The OS was 84.2 ± 8.4% and EFS was 79.0 ± 9.4% with a median follow-up time of 4.3 years (range, 2.6-8.2). The cumulative incidence of CNS relapse was 5.2 ± 5.1%.We conclude that omission of CRT from the treatment of young children with ALL involving the CNS is associated with acceptable survival and avoids potentially devastating late effects in this group.

Background: While low socioeconomic status (SES) has been associated with inferior cancer outcome among adults, its impact in pediatric oncology is unclear. Our objective was therefore to conduct a systematic review to determine the impact of SES upon outcome in children with cancer. Methods: We searched Ovid Medline, EMBASE and CINAHL from inception to December 2012. Studies for which survival-related outcomes were reported by socioeconomic subgroups were eligible for inclusion. Two reviewers independently assessed articles and extracted data. Given anticipated heterogeneity, no quantitative meta-analyses were planned a priori. Results: Of 7,737 publications, 527 in ten languages met criteria for full review; 36 studies met final inclusion criteria. In low- and middle-income countries (LMIC), lower SES was uniformly associated with inferior survival, regardless of the measure chosen. The majority of associations were statistically significant. Of 52 associations between socioeconomic variables and outcome among high-income country (HIC) children, 38 (73.1%) found low SES to be associated with worse survival, 15 of which were statistically significant. Of the remaining 14 (no association or high SES associated with worse survival), only one was statistically significant. Both HIC studies examining the effect of insurance found uninsured status to be statistically associated with inferior survival. Conclusions: Socioeconomic gradients in which low SES is associated with inferior childhood cancer survival are ubiquitous in LMIC and common in HIC. Future studies should elucidate mechanisms underlying these gradients, allowing the design of interventions mediating socioeconomic effects. Targeting the effect of low SES will allow for further improvements in childhood cancer survival. © 2014 Gupta et al.

A premature baby girl born at 28 weeks’ gestation developed thrombocytopenia at approximately 50 days of life (corrected gestational age >35 weeks), which was noted on routine blood work during her admission to the neonatal intensive care unit (NICU). The mother’s pregnancy history was significant for limited pre-natal care. Routine serological testing for rubella, varicella, syphilis, hepatitis B and HIV was not performed, although HIV and hepatitis testing had been normal two years previously. Group B streptococcus status was negative. There was no history of maternal thrombocytopenia. The baby was born at 28 weeks’ gestation via spontaneous vaginal delivery at a peripheral hospital. The infant’s birth weight was 935 g. She was intubated at delivery for low Apgar scores, and transferred to a tertiary care institution for ongoing care. Ongoing neonatal issues at the time of presentation included a persistent oxygen requirement, apnea and bradycardia of prematurity, and poor growth despite fortified feeds. There were intermittent stools positive for occult blood, but no x-ray evidence of necrotizing enterocolitis or feed intolerance. The patient’s complete blood count had been normal at birth, one week of life and four weeks of life. However, the subsequent test at two months of life revealed normocytic anemia and moderate thromobocytopenia. Given the new finding, empirical treatment with broad-spectrum intravenous antibiotics was started for presumed infection. Platelet transfusion was given with an appropriate, albeit transient rise in platelet count. Physical examination was remarkable for mild pallor and palpable cervical lymph nodes in an otherwise well-appearing, nondysmorphic, but small-for-corrected gestational age newborn. Investigations including a complete blood count showed normocytic anemia (hemoglobin level 78 g/L); normal white blood cell count with atypical lymphocytes; and a platelet count of 15×109/L. Platelet reticulocytes were elevated. Liver enzyme levels were mildly elevated, with aspartate aminotransferase 109 U/L and alanine aminotransferase 45 U/L. Echocardiogram and head, abdominal and extremity ultrasounds were unremarkable, with no evidence of thrombus, acute hemorrhage or splenomegaly. Initial infectious work-up, including blood and urine cultures; Epstein-Barr virus serology; urine cytomegalovirus; and hepatitis A, B and C screen, were negative. Rubella serology was indeterminate. An additional blood test confirmed the diagnosis.