Irinotecan and temozolomide in combination with dasatinib and rapamycin versus irinotecan and temozolomide for patients with relapsed or refractory neuroblastoma (RIST-rNB-2011): a multicentre, open-label, randomised, controlled, phase 2 trial.

Background: Neuroblastoma is the most common extracranial solid tumour in children. Relapsed or refractory neuroblastoma is associated with a poor outcome. We assessed the combination of irinotecan-temozolomide and dasatinib-rapamycin (RIST) in patients with relapsed or refractory neuroblastoma.

Trajectories of illness perceptions in paediatric cancer patients and their parents and associations with health-related quality of life: Results of a prospective-longitudinal study.

Objective: In paediatric oncology, little is known about trajectories of illness perceptions and their longitudinal associations with health-related quality of life (HRQoL). Therefore, the aim of this study was to investigate changes in illness perceptions in children and parents over a one-year-period and to investigate predictive value of child's and parent's illness perceptions during acute treatment for child's HRQoL 1 year later.

Methods: N = 65 child-parent-dyads participated in a longitudinal study (retention rate: 80.

Illness perceptions, fear of progression and health-related quality of life during acute treatment and follow-up care in paediatric cancer patients and their parents: a cross-sectional study.

Background: This study examines the role of illness perceptions and fear of progression (FoP) in paediatric cancer patients and their parents for patient's health-related quality of life (HRQoL), controlling for sociodemographic and medical variables. 4-18-year-old patients in acute treatment or follow-up care and one parent were examined.

Methods: N = 46 patient-parent dyads in acute treatment and n = 84 dyads in follow-up care completed measures on illness perceptions (Illness-Perceptions-Questionnaire for 12-18-year-old patients and parents or as age-adapted puppet interview for 4-11-year-old patients) and FoP (Fear-of-Progression-Questionnaire for 7-18-year-old patients and parents).

Epidemiology and Characteristics of Gastric Carcinoma in Childhood-An Analysis of Data from Population-Based and Clinical Cancer Registries.

(1) Background: Gastric carcinoma is an exceptionally rare tumor in childhood. Little is known about the etiology, epidemiology, and clinical features of pediatric gastric carcinomas. This analysis aimed to fill this gap by increasing knowledge about the occurrence of gastric carcinoma in childhood.

Association between Intraoperative Blood Transfusion, Regional Anesthesia and Outcome after Pediatric Tumor Surgery for Nephroblastoma.

Background: Recent data suggest that anesthesiologic interventions-e.g., the choice of the anesthetic regimen or the administration of blood products-might play a major role in determining outcome after tumor surgery.

Multimodal Treatment of Nasopharyngeal Carcinoma in Children, Adolescents and Young Adults-Extended Follow-Up of the NPC-2003-GPOH Study Cohort and Patients of the Interim Cohort.

Nasopharyngeal carcinoma (NPC) in children and young adults has been treated within two consecutive prospective trials in Germany, the NPC-91 and the NPC-2003 study of the German Society of Pediatric Oncology and Hematology (GPOH). In these studies, multimodal treatment with induction chemotherapy, followed by radio (chemo)therapy and interferon-beta maintenance, yielded promising survival rates even after adapting total radiation doses to tumor response. The outcome of 45 patients in the NPC-2003 study was reassessed after a median follow-up of 85 months.

Illness perceptions in patients and parents in paediatric oncology during acute treatment and follow-up care.

Objective: Recent evidence suggests that illness perceptions in paediatric patients and their parents may differ, with parents holding more negative views compared to their children. Little is known about illness perceptions of very young patients and their parents. This study investigates illness perceptions in paediatric cancer patients aged 4-18 years and their parents in acute treatment or follow-up care, distinguishing patients by age (4-11, 12-18) and stage of medical treatment.

Genetic Alterations and Resectability Predict Outcome in Patients with Neuroblastoma Assigned to High-Risk Solely by Amplification.

Background: To identify variables predicting outcome in neuroblastoma patients assigned to the high-risk group solely by the presence of oncogene amplification (MNA).

Methods: Clinical characteristics, genomic information, and outcome of 190 patients solely assigned to high-risk neuroblastoma by MNA were analyzed and compared to 205 patients with stage 4 neuroblastoma aged ≥18 months with MNA (control group).

Results: Event-free survival (EFS) and overall survival (OS) at 10 years were 47% (95%-CI 39-54%) and 56% (95%-CI 49-63%), respectively, which was significantly better than EFS and OS of the control group (EFS 25%, 95%-CI 18-31%, < 0.

A minigene DNA vaccine encoding peptide epitopes derived from Galectin-1 has protective antitumoral effects in a model of neuroblastoma.

We recently identified Galectin-1 (Gal-1), a β-galactoside-binding lectin, as a novel immune regulator in neuroblastoma (NB). Here, we characterized the tolerogenic function of Gal-1 within the CD8 T cell compartment and further evaluated its relevance as an antigen for effective DNA vaccination against NB in a mouse model. NB cells with Gal-1 knockdown (NXS-2L) exhibited significantly reduced tumor growth compared to NXS-2 NB cells.

Clinical and molecular characterization of patients with stage 4(M) neuroblastoma aged less than 18 months without MYCN amplification.

Introduction: The survival of children with stage 4(M) neuroblastoma without MYCN amplification and below the age of 18 months is considered better than the still dismal outcome of older high-risk neuroblastoma patients. This study analyzes the impact of clinical and molecular characteristics on the long-term outcome.

Patients And Methods: Clinical presentation, survival, and recurrence patterns of patients enrolled onto trials NB90, NB97, and NB2004 were retrospectively analyzed.

Psychometric Properties of the Fear of Progression Questionnaire for Children (FoP-Q-SF/C).

Background: Recent research shows that parents of children suffer from fear of progression (FoP), the fear of further disease progression. It is most possible that children also develop FoP, which could impair treatment and psychological health. The aim of this study is to adapt the adult's version of the Fear of Progression Questionnaire - Short Form (FoP-Q-SF) for children and to examine the psychometric properties in pediatric cancer patients.

Results of CoALL 07-03 study childhood ALL based on combined risk assessment by in vivo and in vitro pharmacosensitivity.

We conducted a clinical trial and report the long-term outcome of 773 children with acute lymphoblastic leukemia upon risk-adapted therapy accrued in trial CoALL 07-03 (from the Cooperative Study Group for Childhood Acute Lymphoblastic Leukemia). In a 2-step stratification, patients were allocated to receive either low- or high-risk treatment, based on initial white blood cell count, age, and immunophenotype. A second stratification was performed according to the results of in vitro pharmacosensitivity toward prednisolone, vincristine, and asparaginase (PVA score) and in vivo response after induction therapy (minimal residual disease [MRD]).

Individual and Dyadic Coping and Fear of Progression in Mothers and Fathers of Children with Hematologic Cancer.

This study investigated the association of individual and dyadic coping strategies with fear of progression (FoP) in mothers and fathers of children with hematologic cancer. Parental couples (N = 44) whose children had been diagnosed with hematologic cancer were recruited at a university hospital and a rehabilitation clinic in Germany between 03/2017 and 08/2017. Data included parents' self-report on FoP (Fear of Progression Questionnaire-parent version, FoP-Q-SF/PR), individual coping (Coping Health Inventory for Parents, CHIP-D), and dyadic coping (Dyadic Coping Inventory, DCI).

[Physical Activity Promotion and Exercise in Pediatric Oncology - Structural Requirements and Financing Options Based on the Leipzig Movement Concept].

Regular physical activity is an important requirement for the development and the general health in childhood and adolescence. However, patients during and after treatment for childhood cancer show high levels of physical inactivity, and only a marginal extent of physical activity. In addition to the negative side effects of treatment, this lack of physical activity and exertion has further negative implications for their health, such as decrease in physical performance and health related quality of life.

Daunorubicin during delayed intensification decreases the incidence of infectious complications - a randomized comparison in trial CoALL 08-09.

Anthracyclines are integral components of antileukemic treatment. Apart from cardiotoxicity, myelosuppression and infectious complications have been described for doxorubicin (DOX) and daunorubicin (DNR) as predominant side effects, but little is known about their differential toxicities. To address the question whether DNR is associated with a lower rate of infectious complications compared with DOX, 307 children with newly diagnosed acute lymphoblastic leukemia, enrolled in trial CoALL 08-09, were randomized to receive either DOX 30 mg/m (n = 153) or DNR 36 mg/m (n = 154) in delayed intensification.

Fear of Progression in Parents of Children with Cancer: Results of An Online Expert Survey in Pediatric Oncology.

Background: Fear of Progression (FoP) is a commonly reported psychological strain in parents of children with cancer. This expert survey investigates how professionals in pediatric oncology estimate the burden and consequences of FoP in parents and how they assess and treat parental FoP.

Method: N=77 professionals in pediatric oncology (members and associates of the Psychosocial Association in Paediatric Oncology and Haematology, PSAPOH) were examined in an online survey with a self-developed questionnaire.

Psychometric properties of the Fear of Progression Questionnaire for parents of children with cancer (FoP-Q-SF/PR).

Objective: Psychometric properties of the Fear of Progression Questionnaire - Short Form (FoP-Q-SF) were shown to be good in samples of adult cancer patients and their partners but have so far not been investigated in parents of children with cancer. This study therefore aimed to examine psychometric properties of the previously adapted parent version of the Fear of Progression Questionnaire (FoP-Q-SF/PR) in pediatric oncology.

Methods: N=181 parents (119 mothers, 62 fathers) of n=128 children with diverse cancer entities, up to ten years after diagnosis were recruited at six hospitals and six registered parent associations in Germany and Austria between 06/2015 and 05/2016 (cross-sectional design).

Managing of oral medicines in paediatric oncology: can a handbook and a pharmaceutical counselling intervention for patients and their parents prevent knowledge deficits? A pilot study.

Objectives: To assess knowledge deficits of patients/parents and prevention strategies.

Methods: After receiving ethics approval, we performed a controlled, quasi-randomised, prospective intervention study. We enrolled patients/parents involved in managing oral medicines in three groups: control (routine care only), handbook intervention and pharmaceutical counselling intervention group.

Targeting of heme oxygenase-1 as a novel immune regulator of neuroblastoma.

Heme oxygenase (HO)-1 catalyzes the degradation of cytotoxic heme into biliverdin and blocks antitumor immune responses, thus protecting cancer against host defense. Whether this scenario also applies to neuroblastoma (NB), the most common extracranial solid childhood tumor, is not known. Here, we demonstrate for the first time a prognostic relevance of HO-1 expression in samples from NB patients and show that targeting of HO-1 prevents both cancer resistance against cellular stress and immune escape in the syngeneic NXS2 A/J mouse model of NB.

Signs and symptoms of pediatric brain tumors and diagnostic value of preoperative EEG.

In pediatric patients, brain tumors have been estimated to be the cause for seizures in only 0.2-0.3% of cases, whereas seizures occurred in about 13% of pediatric brain tumor patients at presentation.

Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected].

Pediatric tumors of the central nervous system composed of oligoid tumor cells showing diffuse leptomeningeal spread without a primary mass lesion seem to represent a novel tumor entity. The terms "diffuse leptomeningeal glioneural tumor" or-preferably-"disseminated oligodendroglial-like leptomeningeal tumor of childhood" (DOGLT) were proposed. Four patients were identified with clinico-neuropathologic findings compatible with DOGLT and a mean follow-up time of 54 months was determined.

Integrated PET/MRI for planning navigated biopsies in pediatric brain tumors.

Introduction: An integrated PET/MRI scanner has been used in selected cases of pediatric brain tumor patients to obtain additional metabolic information about lesions for preoperative biopsy planning and navigation.

Patients And Methods: Four patients, age 9-16 years, received PET/MRI scans employing [(11)C]methionine positron emission tomography (PET) and contrast-enhanced 3D-MR sequences for neuronavigation. PET and MR sequences have been matched for neurosurgical guidance.

Salmonella SL7207 application is the most effective DNA vaccine delivery method for successful tumor eradication in a murine model for neuroblastoma.

Attenuated Salmonella is an approved oral life vaccine that is currently entering pre-clinical cancer vaccination studies as a promising DNA carrier. In a syngeneic mouse model for neuroblastoma, oral gavage of Salmonella typhimurium (SL7207) carrying recent generated survivin DNA vaccines induced a stronger cellular anti-NB immune response than gene gun application or injection of lentivirally transduced bone marrow-derived DCs. The level of Salmonella-associated side effects was not significant as indicated by unaffected survivin-mediated hematopoiesis and wound healing.