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Background: A critical aspect of pediatric palliative care consultations is the assessment and documentation of patient and family needs. While these assessments usually include a focus on physical pain, there is less standardization of assessments of other physical symptoms and psychosocial, emotional, or spiritual needs., Aims: To improve the breadth of assessment of psychosocial and emotional needs, screen for symptoms other than pain among pediatric patients utilizing palliative care services, and to increase documentation of assessment data from 30%-40% to 80% through practice changes implemented in 2 Plan-Do-Study-Act (PDSA) cycles., Methods: This quality improvement project involved implementing provider education and adapting the palliative care consultation template in the electronic health record to improve breadth and consistency of assessment and documentation during consultations by the interdisciplinary pediatric palliative care team. Two PDSA cycles were performed. Chi squared tests and statistical control charts were used for data analysis., Results: There was statistically significant improvement in the inclusion of documentation of a pediatric palliative care social work note from baseline (32%) to Cycle 2 (57%). Physical symptom screening declined slightly, but not significantly (p = .32) and socio-emotional discussions also declined but not significantly (p = .05)., Conclusions: Screening for physical symptoms and discussions with patients and families about psychosocial/emotional needs during the initial palliative care consultations are extremely important in providing effective, holistic, patient-centered care. There is a need for development of pediatric-centric guidelines and quality measures to evaluate pediatric palliative care programs; further research is indicated to determine methods for evaluating compliance with these guidelines.

Purpose: Although functional differences have been described between patients with lower extremity bone sarcoma with amputation and limb-preservation surgery, differences have not clearly been shown between the two groups related to quality of life. The purpose of the study was to determine if there is a difference in overall quality of life in lower extremity bone sarcoma survivors related to whether they had an amputation or a limb-preservation procedure while identifying psychological differences for further evaluation. The main hypothesis was that sparing a person's limb, as opposed to amputating it, would result in a better quality of life., Patients and Methods: Eighty-two long-term survivors of lower extremity bone sarcoma were studied to make a comparison of the overall quality of life, pain assessment, and psychological evaluations in limb preservation and amputation patients. Forty-eight patients with limb preservation and thirty-four patients with amputations were enrolled in the study. Validated psychometric measures including the Quality of Life Questionnaire (QLQ), the Minnesota Multiphasic Personality Inventory, and visual analog scales were utilized., Results: The overall quality of life of patients with limb preservation was significantly higher than patients with amputation (p-value < 0.01). Significant differences were noted in the categories of material well-being, job satisfiers, and occupational relations., Conclusion: The overall quality of life of patients with limb-preservation appears to be better than for those patients with amputation based on the QLQ in patients surviving lower extremity bone sarcoma. Further analysis needs to verify the results and focus on the categories that significantly affect the overall quality of life.

Brainstem gliomas (BSG) are a rare group of central nervous system tumors that arise mostly in children and usually portend a particularly poor prognosis. We report the development of a genetically engineered mouse model of BSG using the RCAS/tv-a system and its implementation in preclinical trials. Using immunohistochemistry, we found that platelet-derived growth factor (PDGF) receptor alpha is overexpressed in 67% of pediatric BSGs. Based on this observation, we induced low-grade BSGs by overexpressing PDGF-B in the posterior fossa of neonatal nestin tv-a mice. To generate high-grade BSGs, we overexpressed PDGF-B in combination with Ink4a-ARF loss, given that this locus is commonly lost in high-grade pediatric BSGs. We show that the likely cells of origin for these mouse BSGs exist on the floor of the fourth ventricle and cerebral aqueduct. Irradiation of these high-grade BSGs shows that although single doses of 2, 6, and 10 Gy significantly increased the percent of terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL)-positive nuclei, only 6 and 10 Gy significantly induce cell cycle arrest. Perifosine, an inhibitor of AKT signaling, significantly induced TUNEL-positive nuclei in this high-grade BSG model, but in combination with 10 Gy, it did not significantly increase the percent of TUNEL-positive nuclei relative to 10 Gy alone at 6, 24, and 72 hours. Survival analysis showed that a single dose of 10 Gy significantly prolonged survival by 27% (P = 0.0002) but perifosine did not (P = 0.92). Perifosine + 10 Gy did not result in a significantly increased survival relative to 10 Gy alone (P = 0.23). This PDGF-induced BSG model can serve as a preclinical tool for the testing of novel agents.