Purpose: Long-term survival for children with diffuse intrinsic pontine glioma (DIPG) is less than 10%, and new therapeutic targets are urgently required. We evaluated a large cohort of DIPGs to identify recurrent genomic abnormalities and gene expression signatures underlying DIPG. Patients and Methods: Single-nucleotide polymorphism arrays were used to compare the frequencies of genomic copy number abnormalities in 43 DIPGs and eight low-grade brainstem gliomas with data from adult and pediatric (non-DIPG) glioblastomas, and expression profiles were evaluated using gene expression arrays for 27 DIPGs, six low-grade brainstem gliomas, and 66 nonbrainstem low-grade gliomas. Results: Frequencies of specific large-scale and focal imbalances varied significantly between DIPGs and nonbrainstem pediatric glioblastomas. Focal amplifications of genes within the receptor tyrosine kinase–Ras–phosphoinositide 3-kinase signaling pathway were found in 47% of DIPGs, the most common of which involved PDGFRA and MET. Thirty percent of DIPGs contained focal amplifications of cell-cycle regulatory genes controlling retinoblastoma protein (RB) phosphorylation, and 21% had concurrent amplification of genes from both pathways. Some tumors showed heterogeneity in amplification patterns. DIPGs showed distinct gene expression signatures related to developmental processes compared with nonbrainstem pediatric high-grade gliomas, whereas expression signatures of low-grade brainstem and nonbrainstem gliomas were similar. Conclusion: DIPGs comprise a molecularly related but distinct subgroup of pediatric gliomas. Genomic studies suggest that targeted inhibition of receptor tyrosine kinases and RB regulatory proteins may be useful therapies for DIPG.

Purpose: When a child's cancer progresses beyond current treatment capability, the parents are likely to participate in noncurative treatment decision making. One factor that helps parents to make these decisions and remain satisfied with them afterward is deciding as they believe a good parent would decide. Because being a good parent to a child with incurable cancer has not been formally defined, we conducted a descriptive study to develop such a definition. Methods: In face-to-face interviews, 62 parents who had made one of three decisions (enrollment on a phase I study, do not resuscitate status, or terminal care) for 58 patients responded to two open-ended questions about the definition of a good parent and about how clinicians could help them fulfill this role. For semantic content analysis of the interviews, a rater panel trained in this method independently coded all responses. Inter-rater reliability was excellent. Results: Among the aspects of the definition qualitatively identified were making informed, unselfish decisions in the child's best interest, remaining at the child's side, showing the child that he is cherished, teaching the child to make good decisions, advocating for the child with the staff, and promoting the child's health. We also identified 15 clinician strategies that help parents be a part of making these decisions on behalf of a child with advanced cancer. Conclusion: The definition and the strategies may be used to guide clinicians in helping parents fulfill the good parent role and take comfort afterward in having acted as a good parent.

Approximately 12 400 children are diagnosed with cancer each year in the United States.¹ Regardless of the outcome, cancer and its treatment often take a significant toll on the quality of life of pediatric oncology patients. While the need for palliative care (PC) in this patient population at the end of life, as well as its benefits, have been well established,^2,3 availability and utilization remain low.⁴ Experts in the field have made the recommendation that PC should be introduced at diagnosis and incorporated into care throughout the course of cancer care,^5-7 but in most practices, this does not occur. The American Academy of Pediatrics recommends “an integrated model of PC ‘in which the components of PC are offered at diagnosis and continued throughout the course of illness, whether the outcome ends in cure or death’”^8(p352) for all children with life-limiting illness. Early integration of PC with oncology care for pediatric patients has the potential to improve patients’ physical and emotional well-being as well as their quality of life throughout the course of therapy and beyond.⁷

Background: Adolescent and young adult oncology (AYAO) patients often receive intensive medical care and experience significant symptoms at the end of life (EOL). Objective: This study aimed to describe the characteristics of AYAO patients aged 15–26 years who died as inpatients in a hospital and to compare the illness and EOL experiences of AYAO patients who did and did not receive palliative care (PC). Design and Setting: A standardized data extraction tool was used to collect information about demographics, treatment, terminal characteristics, and symptoms during the last month of life (LMOL) for 69 AYAO patients who died while hospitalized between 2008 and 2014. Measurements and Results: AYAO patients who died in the hospital required considerable medical and psychosocial care and experienced numerous symptoms during the LMOL. Compared to those patients who received no formal PC services, patients followed by the PC team were less likely to die in the intensive care unit (ICU) (38% vs. 68%, p = 0.024) and less likely to have been on a ventilator (34% vs. 63%, p = 0.028) during the LMOL. They also received fewer invasive medical procedures during the LMOL (median, 1 vs. 3 procedures, p = 0.009) and had a do not resuscitate order in place for a longer time before death (median, 6 vs. two days, p = 0.008). Conclusions: Involvement of the PC team was associated with the receipt of less intensive treatments and fewer deaths in the ICU.

The NCCN Guidelines for Palliative Care provide interdisciplinary recommendations on palliative care for patients with cancer. These NCCN Guidelines Insights summarize and provide context for the updated guidelines recommendations regarding hospice and end-of-life (EOL) care. Updates for 2017 include revisions to and restructuring of the algorithms that address important EOL concerns. These recommendations were revised to provide clearer guidance for oncologists as they care for patients with cancer who are approaching the transition to EOL care. Recommendations for interventions and reassessment based on estimated life expectancy were streamlined and reprioritized to promote hospice referrals and improved EOL care.