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Neuroblastoma is the most common extracranial solid tumor diagnosed in children. This inaugural version of the NCCN Guidelines for Neuroblastoma provides recommendations for the diagnosis, risk classification, and treatment of neuroblastoma. The information in these guidelines was developed by the NCCN Neuroblastoma Panel, a multidisciplinary group of representatives with expertise in neuroblastoma, consisting of pediatric oncologists, radiologists, pathologists, surgeons, and radiation oncologists from NCCN Member Institutions. The evidence-based and consensus recommendations contained in the NCCN Guidelines are intended to guide clinicians in selecting the most appropriate treatments for their patients with this clinically heterogeneous disease.

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BACKGROUND: There is growing interest among pediatric institutions for implementing iodine-131 (I-131) meta-iodobenzylguanidine (MIBG) therapy for treating children with high-risk neuroblastoma. Due to regulations on the medical use of radioactive material (RAM), and the complexity and safety risks associated with the procedure, a multidisciplinary team involving radiation therapy/safety experts is required. Here, we describe methods for implementing pediatric I-131 MIBG therapy and evaluate our program's robustness via failure modes and effects analysis (FMEA). METHODS: We formed a multidisciplinary team, involving pediatric oncology, radiation oncology, and radiation safety staff. To evaluate the robustness of the therapy workflow and quantitatively assess potential safety risks, an FMEA was performed. Failure modes were scored (1-10) for their risk of occurrence (O), severity (S), and being undetected (D). Risk priority number (RPN) was calculated from a product of these scores and used to identify high-risk failure modes. RESULTS: A total of 176 failure modes were identified and scored. The majority (94%) of failure modes scored low (RPN <100). The highest risk failure modes were related to training and to drug-infusion procedures, with the highest S scores being (a) caregivers did not understand radiation safety training (O = 5.5, S = 7, D = 5.5, RPN = 212); (b) infusion training of staff was inadequate (O = 5, S = 8, D = 5, RPN = 200); and (c) air in intravenous lines/not monitoring for air in lines (O = 4.5, S = 8, D = 5, RPN = 180). CONCLUSION: Through use of FMEA methodology, we successfully identified multiple potential points of failure that have allowed us to proactively mitigate risks when implementing a pediatric MIBG program.

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Purpose: To test the Clustered Cardiometabolic Risk (CCMR) factor explaining the relationship between physical activity and physical quality of life (QOL). Methods: Using the U.S. National Health and Nutrition Examination Survey 2003-2006, 2,445 adults completed the CDC Healthy Days Questionnaire for measuring QOL, wore the accelerometer for assessing physical activity pattern (PAP), and completed triglyceride, glucose, serum insulin, waist circumference, blood pressure, and HDL-cholesterol tests from which the CCMR factor was created. Physical QOL was classified as poor (≥14 days with poor physical health within past 30 days) vs. good (<14 days). We classified PAP by moderate-to-vigorous physical activity (MVPA), light-intensity physical activity (LIPA), and sedentary behavior (SB). We defined MVPA, LIPA, and SB as ≥2020 counts/minute, 100-2019 counts/minute, and ≤99 counts/minute, respectively. We further classified PAP status as unhealthy (MVPA <150 min/week & SB>LIPA) or healthy (MVPA <150 min/week & SBLIPA or SB

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BackgroundThere is strong evidence of a genetic contribution to Wilms tumor, such as WT1 gene variation or epigenetic changes at chromosome locus 11p15. A previous genome wide association study (GWAS) of Wilms tumor identified other significant association loci including Xp22. Case report: A 4-year-old girl developed a Wilms tumor of the left isthmus of a horseshoe kidney. Chromosomal microarray analysis (CMA) of peripheral blood showed a 563 kb copy number gain at Xp22.11 that included PRDX4 and ZFX. PRDX4 has been shown to play an active role in the tumorigenesis of malignant neoplasms in various organs. Beckwith-Wiedemann methylation analysis and WT1 sequencing were negative. Whole exome sequencing of peripheral blood revealed pathogenic variant in PMS2 gene (c.765C > A), which is consistent with Lynch syndrome. Conclusion: We report a case of Wilms tumor with germline Xp22.11 duplication which further supports this locus as germline susceptibility alteration for Wilms Tumor.

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BACKGROUND: Clostridioides difficile infection (CDI) is a significant source of morbidity in pediatric cancer patients. Few reports to date have evaluated risk factors and short-term outcomes for this population. METHODS: We retrospectively evaluated pediatric oncology admissions at St Louis Children's Hospital from 2009 to 2018. All inpatient cases of diagnosed initial CDI were identified. We aimed to investigate the prevalence of CDI and associated risk factors, including coadmission with another patient with CDI, and to evaluate short-term outcomes including length of stay and delays in subsequent scheduled chemotherapy. RESULTS: Review of 6567 admissions from 952 patients revealed 109 CDI cases (11.4% of patients). Patients with leukemia or lymphoma, compared to those with solid tumors, were more likely to have CDI (odds ratio [OR], 3 [95% CI, 1.4-6.6], and 3 [95% CI, 1.3-6.8], respectively). Autologous hematopoietic stem cell transplant (HSCT) was also a risk factor (OR, 3.5 [95% CI, 1.7-7.4]). Prior antibiotic exposure independently increased the risk for CDI (OR, 3.0 [95% CI, 1.8-4.8]). Concurrent admission with another patient with CDI also significantly increased the risk (OR, 84.7 [95% CI, 10.5-681.8]). In contrast to previous reports, exposure to acid-suppressing medications decreased the risk for CDI (OR, 0.5 [95% CI, .3-.7]). CDI was associated with increased length of stay (mean difference, 8 days [95% CI, 4.6-11.4]) and prolonged delays for subsequent chemotherapy (mean difference, 1.4 days [95% CI, .1-2.7]). CONCLUSIONS: CDI in pediatric oncology patients significantly prolongs hospitalization and delays chemotherapy treatment plans. Interventions to control CDI will improve the care of pediatric oncology patients.

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BACKGROUND: Pediatric hematology/oncology (PHO) patients receiving therapy or undergoing hematopoietic stem cell transplantation (HSCT) often require a central line and are at risk for bloodstream infections (BSI). There are limited data describing outcomes of BSI in PHO and HSCT patients. METHODS: This is a multicenter (n = 17) retrospective analysis of outcomes of patients who developed a BSI. Centers involved participated in a quality improvement collaborative referred to as the Childhood Cancer and Blood Disorder Network within the Children's Hospital Association. The main outcome measures were all-cause mortality at 3, 10, and 30 days after positive culture date; transfer to the intensive care unit (ICU) within 48 hours of positive culture; and central line removal within seven days of the positive blood culture. RESULTS: Nine hundred fifty-seven BSI were included in the analysis. Three hundred fifty-four BSI (37%) were associated with at least one adverse outcome. All-cause mortality was 1% (n = 9), 3% (n = 26), and 6% (n = 57) at 3, 10, and 30 days after BSI, respectively. In the 165 BSI (17%) associated with admission to the ICU, the median ICU stay was four days (IQR 2-10). Twenty-one percent of all infections (n = 203) were associated with central line removal within seven days of positive blood culture. CONCLUSIONS: BSI in PHO and HSCT patients are associated with adverse outcomes. These data will assist in defining the impact of BSI in this population and demonstrate the need for quality improvement and research efforts to decrease them.

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A chemotherapy roadmap is a summary of the chemotherapy plan for a pediatric oncology patient. Chemotherapy roadmaps exist as paper documents for most, if not all, pediatric oncology programs. Paper chemotherapy roadmaps are associated with risks that can negatively affect the safety of the chemotherapy process. This institution explored the feasibility of converting paper chemotherapy roadmaps into an electronic form. The pediatric information systems team developed an innovative computer application that can generate electronic chemotherapy roadmaps, and the pediatric oncology program established a novel workflow that can operationalize them. Electronic chemotherapy roadmaps have been produced for 36 treatment protocols, and 369 electronic chemotherapy roadmaps have been used for 352 pediatric oncology patients. They have functioned as designed and have not had any unintended effects. In the 5 years after their implementation, the average proportion of patient safety events involving paper or electronic chemotherapy roadmaps decreased by 78.7%. This report is the first to demonstrate the feasibility of creating and implementing electronic chemotherapy roadmaps. Continued expansion of the current library will be necessary to formally test the hypothesis that electronic chemotherapy roadmaps can decrease the risks associated with their paper counterparts and increase the safety of the chemotherapy process.

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Burkitt lymphoma presenting as a peritoneal and small thickening problem mimicking carcinomatosis is very rare. We present a previously healthy 7-year-old boy with a 1-week history of worsening abdominal distention, vomiting and melena. He demonstrated liver nodules, but no pathologic chest or abdominal adenopathy was present. It was finally determined that he was suffering from Burkitt lymphoma. We present this case because it is such a rare but very significant manifestation of Burkitt lymphoma.

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The current study describes a system-wide method of evaluating detection strategies for co-occurring disorders within a state mental health system. Information technology was used to merge administrative datasets from the New Jersey mental health and substance abuse systems and identify individuals treated in both systems. We measured a 53% detection rate of substance use disorders amongst adult patients in the mental health system with particularly low detection rates in acute settings (49.0%) and among female (43.7%), older (36.2%), and psychotic patients (43.6%). The methodology described here could help evaluate critical aspects of ongoing state co-occurring disorder quality improvement initiatives.

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OBJECTIVES: We assessed the detection of mental illness in an adult population of substance abuse patients and the rate of referral for mental health treatment. METHODS: We obtained combined administrative records from 1994 to 1997 provided by the New Jersey substance abuse and mental health systems and estimated detection and referral rates of patients with co-occurring disorders (n = 47,379). Mental illness was considered detected if a diagnosis was in the record and considered undetected if a diagnosis was not in the record but the patient was seen in both treatment systems within the same 12-month period. Predictors of detection and referral were identified. RESULTS: The detection rate of co-occurring mental illness was 21.9% (n=10364); 57.9% (n=6001) of these individuals were referred for mental health treatment. Methadone maintenance clinics had the lowest detection rate but the highest referral rate. Male, Hispanic, and African American patients, as well as those who used heroin or were in the criminal justice system, had a higher risk of mental illness not being detected. Once detected, African American patients, heroin users, and patients in the criminal justice system were less likely to be referred for treatment. CONCLUSIONS: There is a need to improve the detection of mental illness among substance abuse patients and to provide integrated treatment.

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OBJECTIVE: The Patient Health Questionnaire depression scale (PHQ-9) is a well-validated, Diagnostic and Statistical Manual of Mental Disorders- Fourth Edition (DSM-IV) criterion-based measure for diagnosing depression, assessing severity and monitoring treatment response. The performance of most depression scales including the PHQ-9, however, has not been rigorously evaluated in different racial/ethnic populations. Therefore, we compared the factor structure of the PHQ-9 between different racial/ethnic groups as well as the rates of endorsement and differential item functioning (DIF) of the 9 items of the PHQ-9. The presence of DIF would indicate that responses to an individual item differ significantly between groups, controlling for the level of depression. MEASUREMENTS: A combined dataset from 2 separate studies of 5,053 primary care patients including non-Hispanic white (n=2,520), African American (n=598), Chinese American (n=941), and Latino (n=974) patients was used for our analysis. Exploratory principal components factor analysis was used to derive the factor structure of the PHQ-9 in each of the 4 racial/ethnic groups. A generalized Mantel-Haenszel statistic was used to test for DIF. RESULTS: One main factor that included all PHQ-9 items was found in each racial/ethnic group with alpha coefficients ranging from 0.79 to 0.89. Although endorsement rates of individual items were generally similar among the 4 groups, evidence of DIF was found for some items. CONCLUSIONS: Our analyses indicate that in African American, Chinese American, Latino, and non-Hispanic white patient groups the PHQ-9 measures a common concept of depression and can be effective for the detection and monitoring of depression in these diverse populations.

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OBJECTIVE: Because Chinese Americans are known to underutilize mental health services, routine screening for depression in primary care clinics has been advocated as a means of identifying Chinese-American patients with depression and initiating appropriate treatment. This analysis evaluated the utility of the nine-item depression module of the Patient Health Questionnaire (PHQ-9) for depression screening and monitoring treatment outcomes among Chinese Americans at a primary health care setting. METHODS: A total of 3,417 patients who presented for an initial or annual physical examination with their primary care providers were screened for depression between January and October 2003 at the Charles B. Wang Community Health Center, a primary health care center in New York City serving primarily low-income Chinese Americans. The depression screen was a two-tier process: if patients endorsed one or more of three initial questions on an abridged version of the PHQ-9, they were then administered the PHQ-9. RESULTS: A total of 141 individuals (4.1 percent) had clinically significant depressive symptoms (PHQ-9 score of 10 or higher). Women were more likely to have significant levels of depressive symptoms than men (17.3 percent compared with 11.6 percent). A total of 114 of the 141 patients with clinically significant depressive symptoms received treatment plans initiated by their primary care provider. Forty (35.1 percent) responded to treatment (50 percent decrease in PHQ-9 score) by eight weeks of treatment. CONCLUSIONS: The PHQ-9 can be used to screen for depression as well as guide depression treatment planning among Chinese Americans in primary care. Screening for major depression with the PHQ-9 in primary care settings should be considered for appropriate identification and treatment of depression for Chinese-American patients.

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OBJECTIVE: Major depression has a negative impact on functional status and quality of life, but little is known about racial or ethnic differences in the relationship between depression and functional disability. This study compared the association between depression severity and functional status among three different racial or ethnic groups. METHODS: Data were from participants in the original Patient Health Questionnaire (PHQ) Primary Care Study and PHQ Obstetrics-Gynecology Study (N=5,427). Among the study participants, 62.0 percent were non-Hispanic white, 14.8 percent were African American, and 23.1 percent were Latino. Depression severity was assessed with the PHQ-9, the self-administered DSM-IV criteria-based depression module of the PHQ diagnostic instrument for mental disorders. Functional status was measured with the scales of the Medical Outcomes Study 20-item Short Form Health Survey (SF-20), self-reported disability days, clinic visits, and symptom-related difficulty. Groups were compared in terms of mean PHQ-9 scores and functional status at varying levels of depression severity. Linear regression was used to control for age, gender, education level, and language. RESULTS: Mean PHQ-9 scores were not significantly different between the three different racial or ethnic groups. Similar linear associations were found in all three racial or ethnic groups between increasing PHQ-9 scores (more severe depression) and worsening function on the SF-20 scales and an increased number of disability days and clinic visits. Latinos reported significantly less functional impairment on all measures of functionality compared with non-Hispanic whites. CONCLUSIONS: Functional impairment increased with increasing levels of depression severity in all three racial or ethnic groups, although Latinos consistently reported fewer functional disturbances compared with non-Hispanic whites.

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This paper outlines the ways in which an individual's notions of sexuality are challenged by immigration. It describes four basic facets of sexuality and the ways in which each are rooted in culture. These include (1) the relative importance of sexuality in daily life; (2) potential or overt homosexuality; (3) marriage-whether based on arrangement or love; and (4) cross-cultural romantic and marital relationships. Immigration can stir up conflicts in each of these realms and lead to defensive retreat into familiar customs or a counter-phasic plunge into new cultural avenues. Ideally, it would lead to a working-through of these conflicts and subsequent growth in the immigrant's understanding of his or her sexual self.

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We describe 2 adolescent boys with facial swelling and/or subcutaneous nodules and fever. Extensive evaluation, including several biopsy specimens, led to a diagnosis of subcutaneous panniculitic T-cell lymphoma, an entity rarely seen in children. Both patients were treated with oral cyclosporine in an effort to suppress the cytokine release from T-cells that has been thought to induce the hemophagocytic syndrome. The patients responded dramatically to cyclosporine treatment with defervescence of the fever and reduction in number and size of the subcutaneous nodules. Subsequent therapy with multidrug chemotherapy achieved complete remission in the first patient. This report suggests the value of cyclosporine as a first-line agent coupled with chemotherapy in the treatment of patients with subcutaneous panniculitic T-cell lymphoma. A clinicopathologic review of 8 described pediatric cases of subcutaneous panniculitic T-cell lymphoma is also presented.

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Several growth factors are trophic for the gastrointestinal tract and able to reduce the degree of intestinal damage caused by cytotoxic agents. However, studies of epidermal growth factor (EGF) for chemotherapy-induced intestinal injury are conflicting. The development of a transgenic mouse that specifically overexpresses EGF in the small intestine provided a unique opportunity to assess the contribution of EGF in mucositis. After a course of fluorouracil, transgenic mice fared no better than control mice. Weight recovery was inferior, and mucosal architecture was not preserved. Apoptosis was not decreased and proliferation was not increased in the crypts. To corroborate the findings in transgenic mice, ICR mice were treated with exogenous EGF after receiving fluorouracil. Despite ileal upregulation of native and activated EGF receptor, the mice were not protected from intestinal damage. No benefits were observed with different EGF doses or schedules or routes of EGF administration. Finally, mucositis was induced in mutant mice with specific defects of the EGF signaling axis. Compared with control mice, clinical and histological parameters of intestinal injury after fluorouracil were no different in waved-2 mice, which have functionally diminished EGF receptors, or waved-1 mice, which lack transforming growth factor-alpha, another major ligand for the EGF receptor. These findings do not support a critical role for EGF or its receptor in chemotherapy-induced intestinal injury.

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