Guideline for the prevention of acute chemotherapy-induced nausea and vomiting in pediatric cancer patients: A focused update.

This update of the 2013 clinical practice guideline provides clinicians with guidance regarding the use of aprepitant and palonosetron for the prevention of acute chemotherapy-induced nausea and vomiting (CINV) in children. The recommendations were based on three systematic reviews. Substantive changes were made to the guideline recommendations including the inclusion of palonosetron to the 5-HT3 antagonists recommended for children receiving highly emetogenic chemotherapy (HEC) and the recommendation of aprepitant for children 6 months of age or older receiving HEC. To optimize CINV control in children, future work must focus on closing critical research gaps.

Contributions of advanced practice nurses with a DNP degree during palliative and end-of-life care of children with cancer.

The doctorate in nursing practice (DNP) degree is recommended as the terminal degree for advanced practice nurses by 2015. Improvement in the quality of palliative and end-of-life care for children with cancer is recognized as a health care priority. The purpose of this article is to describe: (a) how the American Association of Colleges of Nursing's 8 core elements and competencies can be used by DNP-advanced practice nurses in pediatric oncology settings and (b) the DNP-advanced practice nurses' leadership role to advocate translation of evidence in the care of pediatric oncology patients and to promote interdisciplinary collaboration to improve health care outcomes for pediatric oncology patients.

Radiation-induced gliomas in 2 pediatric patients with neurofibromatosis type 1: case study and summary of the literature.

Neurofibromatosis type 1 (NF1) is a genetic disorder that predisposes patients to the formation of sporadic tumors and also increases the risk of radiation-induced malignancies. The most commonly described radiation-induced tumor in NF1 patients is a malignant peripheral nerve sheath tumor. We present 2 children with NF1 who received radiation therapy and subsequently developed high-grade gliomas. We then review the current literature on radiation-induced tumors in NF1 patients. Although radiation may be the most appropriate therapy in specific situations for children with NF1, the secondary tumor risk should be carefully considered.

Development of an in-home standardized end-of-life treatment program for pediatric patients dying of brain tumors.

PURPOSE: To evaluate an end-of-life (EOL) program related to specific outcomes (i.e., number of hospitalizations and place of death) for children with brain tumors. DESIGN AND METHODS: From 1990 to 2005, a retrospective chart review was performed related to specified outcomes for 166 children with admission for pediatric brain tumors. RESULTS: Patients who received the EOL program were hospitalized less often (n = 114; chi-square = 5.001 with df = 1, p <.05) than patients who did not receive the program. PRACTICE IMPLICATIONS: An EOL program may improve symptom management and decrease required hospital admissions for children with brain tumors.

Memory disorders in children with central nervous system germ cell tumors.

Memory disorders in children and adolescents with brain tumors are rare, but devastating to social, academic, and vocational development. Many pediatric patients with intracranial germ cell tumors (GCTs) complain of memory difficulties. This study investigated memory across a series of GCT patients. A total of 33 GCT patients were retrospectively examined for diagnosis, imaging results, intelligence quotient, treatment variables, evidence of increased intracranial pressure at diagnosis, and memory. The incidence of amnesia in GCT patients was 55%. Memory disturbance could not be predicted by intelligence quotient, treatment, location of lesion, or hydrocephalus at diagnosis. The high incidence of memory deficits in GCT patients suggests a risk to memory in patients with GCT. Formal memory assessment should be considered in all patients with central nervous system GCTs. Specific counseling and planning to assist in adjustment and to ensure safety should be considered standard care for those with memory deficits. The nurse should be instrumental in facilitating understanding of this specific injury in the brain tumor population.

Potential for improved intelligence quotient using volumetric modulated arc therapy compared with conventional 3-dimensional conformal radiation for whole-ventricular radiation in children.

PURPOSE: To compare volumetric modulated arc therapy (VMAT) with 3-dimensional conformal radiation therapy (3D-CRT) in the treatment of localized intracranial germinoma. We modeled the effect of the dosimetric differences on intelligence quotient (IQ). METHOD AND MATERIALS: Ten children with intracranial germinomas were used for planning. The prescription doses were 23.4 Gy to the ventricles followed by 21.6 Gy to the tumor located in the pineal region. For each child, a 3D-CRT and full arc VMAT was generated. Coverage of the target was assessed by computing a conformity index and heterogeneity index. We also generated VMAT plans with explicit temporal lobe sparing and with smaller ventricular margin expansions. Mean dose to the temporal lobe was used to estimate IQ 5 years after completion of radiation, using a patient age of 10 years. RESULTS: Compared with the 3D-CRT plan, VMAT improved conformality (conformity index 1.10 vs 1.85), with slightly higher heterogeneity (heterogeneity index 1.09 vs 1.06). The averaged mean doses for left and right temporal lobes were 31.3 and 31.7 Gy, respectively, for VMAT plans and 37.7 and 37.6 Gy for 3D-CRT plans. This difference in mean temporal lobe dose resulted in an estimated IQ difference of 3.1 points at 5 years after radiation therapy. When the temporal lobes were explicitly included in the VMAT optimization, the mean temporal lobe dose was reduced 5.6-5.7 Gy, resulting in an estimated IQ difference of an additional 3 points. Reducing the ventricular margin from 1.5 cm to 0.5 cm decreased mean temporal lobe dose 11.4-13.1 Gy, corresponding to an estimated increase in IQ of 7 points. CONCLUSION: For treatment of children with intracranial pure germinomas, VMAT compared with 3D-CRT provides increased conformality and reduces doses to normal tissue. This may result in improvements in IQ in these children.

Memory deficits in patients with pediatric CNS germ cell tumors.

BACKGROUND: Germ cell tumors (GCT) of the central nervous system including germinomas and nongerminomatous germ cell tumors are rare neoplasms most commonly affecting children and adolescents. Many GCT patients seen at The Children's Hospital Denver complain of memory difficulties at the time of presentation, or demonstrate memory difficulties when assessed. Although case studies suggest that memory deficits may be associated with GCT, this is the first study to investigate memory function across a series of pediatric intracranial GCT patients. PROCEDURE: A total of 26 GCT patients were retrospectively examined for diagnosis, imaging results, intelligence quotient, treatment variables, evidence of increased intracranial pressure at diagnosis, and memory function. Patient Full Scale IQ was measured using the Wechsler intelligence scales. Memory was evaluated with the California Verbal Learning Test. RESULTS: The incidence of amnesia in GCT patients was 42%. GCT patients with amnesia were significantly older at diagnosis than those who did not develop amnesia. There was no association between hydrocephalus at presentation or having received radiation and the presence of memory deficits. Several cases of amnesia were not associated with involvement of classic memory structures. CONCLUSION: The high incidence of measurable memory deficits in GCT patients suggests that amnesia may be a significant risk in this patient population. Memory assessment at diagnosis and appropriate follow-up services may prove beneficial for GCT patients.

Radiation-induced glioblastoma multiforme in children treated for medulloblastoma with characteristics of both medulloblastoma and glioblastoma multiforme.

Outcomes for average-risk medulloblastoma are excellent with 5-year event-free survival and overall survival>80%. Treatment failures include radiation-induced glioblastomas (RIG), which are often diagnosed solely on imaging. Recent studies suggest that RIGs differ from spontaneous glioblastoma multiforme (GBM), based on microarray gene-expression profiling. Retrospective review of children with average-risk medulloblastoma treated from 1996 to 2003 included 16 patients with 5 treatment failures. One died of disease progression, 1 died as a result of radiation necrosis, and 3 children died of pathology-confirmed GBM. Of these 3 GBMs, one was studied with electron microscopy, cytogenetics, and gene-expression microarray analysis. This tumor had focal medulloblastoma and similarity by gene-expression microarray with other RIGs. With both components in the recurrent tumor, we suggest it was in the process of transitioning from medulloblastoma to RIG, that is, "catching the tumor in the act." Some radiation-induced nervous system tumors may develop as a direct result of severe oncologic changes within the original tumor cells, with the tumor evolving into a different phenotypic tumor type. We recommend biopsy for tissue confirmation and genetic expression profile to shed light on the etiology of radiation-induced neoplasms.

Creative arts therapy improves quality of life for pediatric brain tumor patients receiving outpatient chemotherapy.

This mixed methods pilot study evaluated the effects of the creative arts therapy (CAT) on the quality of life (QOL) of children receiving chemotherapy. A 2-group, repeated measures randomized design compared CAT with a volunteer's attention (n = 16). Statistical analysis of the randomized controlled phase of the study suggested an improvement in the following areas after the CAT: parent report of child's hurt (P = .03) and parent report of child's nausea (P = .0061). A nonrandomized phase, using a different instrument showed improved mood with statistical significance on the Faces Scale (P < .01), and patients were more excited (P < .05), happier (P < .02), and less nervous (P < .02). Provider focus groups revealed positive experiences. Case studies are included to exemplify the therapeutic process. With heightened interest in complementary therapy for children with cancer, future research with a larger sample size is needed to document the impact of incorporating creative arts into the healing process.

Experience with electrolyte levels after craniotomy for pediatric brain tumors.

Children with brain tumors routinely undergo craniotomies for tumor resections. Nurses and nurse practitioners are critical in closely monitoring these patients. Postoperatively, these children may develop inappropriate vasopressin secretion, cerebral salt wasting syndrome, or a combination of both. Inappropriate fluid and electrolyte administration may exacerbate symptoms. Both high and low sodium levels are associated with significant complications. Sodium levels were prospectively observed for 72 hours perioperatively. Overall, 36 patients had 201 serum sodium levels measured over the course of 1 year. Postoperatively, 79 (39%) of the sodium levels were <135 mEq/L or >145 mEq/L. Of these abnormal sodium levels, 3 (1%) were <125 mEq/L and 2 (1%) were >165 mEq/L. All the abnormal sodium levels occurred in patients with suprasellar or hypothalamic lesions. This study suggests that children with resections of suprasellar or hypothalamic tumors need specialist attention in postoperative management of fluid and electrolytes. The nurse is in the ideal role to monitor closely the pediatric brain tumor patient at high risk for abnormal sodium levels.

Unique molecular characteristics of radiation-induced glioblastoma.

Radiation-induced glioblastomas (RIGs) represent a significant proportion of glioblastomas (GBMs) seen in children and young adults and manifest poor prognosis. Little is known about their underlying biology, although limited studies have suggested no unique histologic or cytogenetic characteristics to distinguish them from de novo GBMs. In this study, we confirmed that a series of 5 RIGs showed no unique histologic or cytogenetic features compared with de novo pediatric GBMs, prompting us to further investigate RIGs using gene expression microarray profiling and Western blot analysis. Despite the inability of histologic and molecular genetic studies to identify distinguishing features between RIGs and pediatric GBMs, gene microarrays suggested significant differences between these 2 tumor types, at least those occurring in pediatric patients. Pediatric RIGs show greater homogeneity of gene expression than do de novo pediatric GBMs. Greater overlap was detected in gene expression patterns between RIGs and pilocytic astrocytomas than between RIGs and GBMs, medulloblastomas, ependymomas, atypical teratoid rhabdoid tumors, or rhabdomyosarcomas, suggesting a common precursor cell for RIG and pilocytic astrocytoma. Western blot analyses confirmed that ErbB3, Sox10, and platelet-derived growth factor receptor-alpha proteins were consistently expressed in RIGs but rarely in pediatric GBMs.

Molecular array analyses of 51 pediatric tumors shows overlap between malignant intracranial ectomesenchymoma and MPNST but not medulloblastoma or atypical teratoid rhabdoid tumor.

Gene microarray has been used to identify prognostic markers and genes of interest for therapeutic targets; a less common use is to show possible histogenetic relationships between rare tumor types and more common neoplasms. Intracranial malignant ectomesenchymoma (MEM) is a pediatric tumor postulated to arise from neural crest cells that contain divergent neuroectodermal and mesenchymal tissues, principally mature ganglion cells and rhabdomyosarcoma (RMS). We investigated a case of MEM by molecular, cytogenetic, and gene array analyses and compared results with our previously unpublished series of 51 pediatric tumors including conventional RMS, Ewing sarcoma (EWS), medulloblastoma (MED), atypical teratoid rhabdoid tumor (ATRT), and malignant peripheral nerve sheath tumor (MPNST); the latter is a sarcoma also with potential for divergent differentiation. Standard cytogenetic analyses and RT-PCR testing for the classic gene rearrangements seen in RMS [t(2;13)-PAX3/FKHR] and EWS ([t(11;22) & t(21;22)-EWS/FLI-1 & EWS/ERG), were used for characterization of the MEM, with gene expression microarray analyses on all tumor types. Gene rearrangement studies were negative in MEM. Gene expression microarray analyses showed tight clustering of the MEM with the MPNST (n = 2), but divergence from other pediatric tumors. MEM and MPNST both showed complex karyotypes, but without diagnostic translocations. Despite the presence of malignant skeletal muscle differentiation in the MEM, gene array testing showed no overlap with RMS, MED, or ATRT, but rather with MPNST. This suggests a common stem cell origin or embryonic gene recapitulation for these tumors and provides novel insights into their underlying biology.