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Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Purpose: To assess the value of diffusion-weighted MRI (DW-MRI) in the non-invasive prediction of blastemal remnant after neoadjuvant chemotherapy in nephroblastoma. Methods: This IRB-approved study included 32 pediatric patients with 35 tumors who underwent DW-MRI prior and after completion of neoadjuvant chemotherapy and subsequent surgical resection. Two blinded radiologists volumetrically assessed each tumor on pre- and post-neoadjuvant images and the parameters mean ADC, median ADC, 12.5th/25th/75th ADC percentile, skewness, and kurtosis were calculated. Blastemal remnant was determined per the pathology report. Associations between imaging features and blastemal remnant quartiles were examined using the Kruskal–Wallis test and adjusted for false discovery rate. Results: Inter-reader agreement was high for mean ADC, skewness, kurtosis, and volume (ICC: 0.76–0.998). Pre-therapeutic histogram parameters skewness and kurtosis were found to be higher in patients with a higher amount of blastemal remnant for reader 1 (overall p = 0.035) and for kurtosis in reader 2 (overall p = 0.032) with skewness not reaching the level of statistical significance (overall p = 0.055). Higher tumor volume on pre-treatment imaging was associated with a higher amount of blastemal remnant after therapy (overall p = 0.032 for both readers). Conclusions: Pre-treatment skewness and kurtosis of ADC histogram analysis were significantly associated with a larger fraction of a blastemal remnant after neoadjuvant chemotherapy. These findings could be incorporated into a more personalized chemotherapeutic regime in these patients and offer prognostic information at the time of initial diagnosis. [ABSTRACT FROM AUTHOR]

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Der Pathologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Monatsschrift Kinderheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Purpose: To assess the value of diffusion-weighted MRI in the pre-therapeutic evaluation of pediatric renal cortical tumors. Methods: This IRB-approved, retrospective multi-center study included 122 pediatric patients with 130 renal tumors, who underwent MRI including DWI before neoadjuvant chemotherapy and nephrectomy. Two radiologists independently assessed each tumor volumetrically, and apparent diffusion coefficient (ADC) values were calculated on a voxel-wise basis, including parameters derived from histogram and texture analysis. Results: Inter-reader agreement was excellent (ICC 0.717–0.975). For both readers, patients with locally aggressive tumor growth (SIOP 3 stage) or with metastases (M1) had significantly lower 12.5th-percentile ADC values (p ≤ 0.028) compared to those with lower-stage tumors, and the parameter energy differed significantly between patients with M1 and those with M0 status (p ≤ 0.028). Contrast and homogeneity differed significantly between benign nephroblastomatosis and malignant nephroblastoma (p ≤ 0.045, both readers). As compared to all other subtypes, the blastemal subtype demonstrated significantly higher skewness (p ≤ 0.022, both readers) and the diffuse anaplastic subtype demonstrated significantly higher 75th-percentile ADC values (p ≤ 0.042, both readers). Conclusions: Diffusion-weighted MRI may be of value in identifying benign nephroblastomatosis and assessing nephroblastoma subtypes. Therefore, further research is warranted to assess its value in risk stratification for pediatric patients with renal tumors in the future. [ABSTRACT FROM AUTHOR]

Background: Wilms tumor (WT) with an inferior Vena cava (IVC) malignant thrombus comprises 4–10% of all WT cases. Methods: This retrospective analysis included 51 pediatric patients presenting at Children Cancer Hospital Egypt-57357 from July 2007 to December 2016 with the diagnosis of WT with malignant IVC thrombus. Results: Median age at presentation = 4.4 years and 28 cases (55%) were females. Twenty-five patients (49%) were metastatic and 4 patients (7.8%) had bilateral disease. Forty-seven cases (92.2%) had favorable histology with no evidence of anaplasia. Level of thrombus extension at presentation was classified as infra-hepatic, retro-hepatic, supra-hepatic and intra-cardiac in 33, 9, 6 and 3 patients, respectively. Fifty patients started neoadjuvant chemotherapy (CTH) with 16 patients showing complete resolution of thrombus after 6 weeks of CTH. None of the patients developed thrombus progression after neoadjuvant CTH; one patient had stationary intra-cardiac thrombus, while remaining patients showed partial regression of their thrombus and had nephrectomy with en-bloc thrombectomy. The mean cranio-caudal dimension of IVC thrombi at initial presentation was 6.5 cm, and 3.6 cm post 6th week of CTH. The 5-year OS and EFS were 75.9% and 71.1%, respectively. There was no significant correlation of initial levels of thrombus extension with survival. Conclusion: Neoadjuvant chemotherapy followed by radical nephrectomy with en-bloc thrombectomy and radiotherapy seems a successful approach for management of patients with WT and IVC tumor thrombus. Measurement of the cranio-caudal dimension of thrombus and its response to treatment should be considered in the surgical planning. [ABSTRACT FROM AUTHOR]

Copyright of Wiener Medizinische Wochenschrift is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Soft tissue tumors of infancy encompass an overlapping spectrum of diseases that pose unique diagnostic and clinical challenges. We studied genomes and transcriptomes of cryptogenic congenital mesoblastic nephroma (CMN), and extended our findings to five anatomically or histologically related soft tissue tumors: infantile fibrosarcoma (IFS), nephroblastomatosis, Wilms tumor, malignant rhabdoid tumor, and clear cell sarcoma of the kidney. A key finding is recurrent mutation of EGFR in CMN by internal tandem duplication of the kinase domain, thus delineating CMN from other childhood renal tumors. Furthermore, we identify BRAF intragenic rearrangements in CMN and IFS. Collectively these findings reveal novel diagnostic markers and therapeutic strategies and highlight a prominent role of isolated intragenic rearrangements as drivers of infant tumors. [ABSTRACT FROM AUTHOR]

In a German pediatric oncology unit, the attending physicians diagnosed 27 cases of Clostridium difficile-associated disease (CDI) from January 01, 2010 to October 31, 2013. This refers to a CDI incidence density of 2.0/1000 inpatient days. According to the hospital hygiene standard, symptomatic patients with CDI were kept in contact isolation. Most patients (median age 8.2 years) suffered from acute lymphoblastic leukemia; 88.9% were treated with broad-spectrum antibiotics during the preceding 4 weeks. 29.6% received intravenous morphine/metamizole and parenteral nutrition due to severe chemotherapy-induced mucositis. None of the patients experienced severe complications such as lower gastrointestinal tract bleeding, sepsis, or toxic megacolon. Genotyping of the isolates derived from symptomatic patients revealed many different ribotypes without detection of the hypervirulent 027 strain and did not point at hospital transmission as an important promoter of CDI in our unit.Conclusion: Under strict standard hygiene and contact isolation for symptomatic patients, genotyping of clinical isolates revealed that in pediatric cancer patients, CDI is not necessarily based on nosocomial transmission. The rate of CDI-related severe complications was low. What is Known: • Pediatric cancer patients face an increased risk of Clostridium difficile-associated disease due to immunosuppression, cancer chemotherapy, mucositis, and dysbiosis following intravenous broad-spectrum antimicrobial treatment. • C. difficile may be transmitted from patient to patient. What is New: • Under strict standard hygiene and contact isolation for symptomatic patients, genotyping of clinical isolates revealed that in pediatric cancer patients, CDI is not necessarily based on nosocomial transmission. • The rate of CDI-related severe complications was low. [ABSTRACT FROM AUTHOR]

Background: Perioperative antimicrobial prophylaxis (PAP) has been identified as an important target for internal audits, concerning the judicious use of antibiotics. Paediatric oncology patients with brain tumours face an increased risk of surgical site infection (SSI) after neurosurgery and receive routine PAP in this setting. Patients and methods: All patients younger than 18 years admitted to the paediatric oncology centre (POC) with a neurosurgical intervention. Systematic audit of routine clinical data is divided in two groups: retrospective (Jan 01, 2012-March 31, 2014) and prospective (April 01, 2014-March 31, 2015) referring to an internal PAP guideline, invented in Jan. 2014). Surveillance of SSI up to 30 days after the operation with standard criteria (Centres for Disease Control and Prevention, USA). Results: In total, 53 neurosurgical operations were analysed in 33 paediatric oncology patients. Twelve patients received more than one operation. The detailed analysis of PAP revealed prophylactic cefuroxim doses about 30 mg/kg instead of 50 mg/kg and no repeated dosing in operations lasting longer than 4 h. In addition, Cefotaxim, which is not indicated as PAP in neurosurgery, was used instead of Cefuroxim (or Ampicillin-Sulbactam) in 23 % of all cases in the retrospective and 18 % of all cases in the prospective audit. PAP for more than 3 doses (>24 h) was administered in 66 % in the retrospective group and in 60 % in the prospective group ( p = n.s.). In both groups, no SSI was detected. Discussion: This first comprehensive audit of PAP in paediatric oncology patients undergoing neurosurgery outlines significant opportunities to improve clinical practice in terms of correct dosing, the correct choice of the antibiotic, a correct timing schedule and a shorter duration of PAP. In addition, our results illustrate in detail the challenges in clinical practice when an evidence-based approach to improve a standard workflow has to be implemented. [ABSTRACT FROM AUTHOR]

Copyright of Der Onkologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Purely intracranial soft tissue sarcomas (ISTS) are very rare among children. A retrospective database analysis of the Cooperative Weichteilsarkom Studiengruppe (CWS) and brain tumor (HIT) registries was conducted to describe treatment and long-term outcome of children and adolescents with ISTS. Nineteen patients from Germany, Austria and Switzerland were reported between 1988 and 2009. Median age at diagnosis was 9.7 years (range, 0.5-17.8). Central pathological review was performed in 17 patients. Eleven patients underwent a total and five a subtotal tumor resection. A biopsy was done in one patient. In two patients no data concerning extent of initial resection was available. Radiotherapy was performed in 15 patients (first-line, n = 11; following progression, n = 4). All but one patient received chemotherapy (first-line, n = 7, following progression, n = 5; first-line and following progression, n = 6). With a median follow-up of 5.8 years (range, 0.6-19.8) ten patients were alive in either first or second complete remission. Seven patients died due to relapse or progression and two were alive with progressive disease. Estimated progression-free and overall survival at 5 years were 47 % (±12 %) and 74 % (±10 %), respectively. About 50 % of patients with ISTS remain relapse-free after 5 years. Multimodality treatment including complete tumor resection and radio-/chemotherapy is required to achieve sustained tumor control in patients with ISTS. Early initiation of postoperative non-surgical treatment seems to be important to prevent recurrence. Due to the intracranial localization local therapy should follow the recommendations used in brain tumors rather than in soft tissue sarcomas, whereas chemotherapy should be guided by histological subtype. [ABSTRACT FROM AUTHOR]

Nanoinformatics has recently emerged to address the need of computing applications at the nano level. In this regard, the authors have participated in various initiatives to identify its concepts, foundations and challenges. While nanomaterials open up the possibility for developing new devices in many industrial and scientific areas, they also offer breakthrough perspectives for the prevention, diagnosis and treatment of diseases. In this paper, we analyze the different aspects of nanoinformatics and suggest five research topics to help catalyze new research and development in the area, particularly focused on nanomedicine. We also encompass the use of informatics to further the biological and clinical applications of basic research in nanoscience and nanotechnology, and the related concept of an extended 'nanotype' to coalesce information related to nanoparticles. We suggest how nanoinformatics could accelerate developments in nanomedicine, similarly to what happened with the Human Genome and other -omics projects, on issues like exchanging modeling and simulation methods and tools, linking toxicity information to clinical and personal databases or developing new approaches for scientific ontologies, among many others. [ABSTRACT FROM AUTHOR]

Previous results had documented oncolytic capacity of reovirus, parvovirus and Newcastle disease virus (NDV) on several tumor cell types. To test whether combinations of these viruses may increase this capacity, human U87- and U373-glioblastoma cells, in vitro or xenografted into immuno-compromised mice, were subjected to simultaneous double infections and analyzed. Our results show that reovirus (serotype-3) plus NDV (Hitcher-B1) and reovirus plus parvovirus-H1 lead to a significant increase in tumor cell killing in vitro in both cell lines (Kruskal-Wallis test , P < 0.01) and in vivo. Immunofluorescence and flow cytometry analyses demonstrated the simultaneous replication of the viruses in nearly all cells (>95%) after combined infection. These data thus indicate that a synergistic anti-tumor effect can be achieved by the combined infection with oncolytic viruses. [ABSTRACT FROM AUTHOR]

Purpose: Tumor segmentation constitutes a crucial step in simulating cancer growth and response to therapy. Incorporation of imaging data individualizes the simulation and assists clinical correlation with the predicted outcome. We adapted snakes to improve tumor segmentation including difficult cases with inherently inhomogeneous structure and poorly defined margins. Methods: Snakes are flexible curves, based on the parameter-controlled deformation of an initial user-defined contour toward the boundary of the desired object, through the minimization of a suitable energy function. Although parameter-adjustment can yield fairly good results in homogeneous regions, traditional snakes often fail to provide an accurate segmentation result when both rigid and very elastic behavior is needed simultaneously to delineate the true outline of the tumor. We developed and tested a spatially adaptive active contour technique by introducing local snake bending, to improve traditional snakes performance for segmenting tumors. The key point of our method is the use of adaptable snake parameters, instead of constant ones, to adjust the bending of the curve according to the local edge characteristics. Our algorithm discriminates image regions according to underlying image features, such as gradient magnitude and corner strength. More specifically, it assigns each region a different 'localized' set of parameters, one corresponding to a very flexible snake, and the other corresponding to a very rigid one, according to the local image characteristics. Results: Qualitative results on more than 150 real MR images, as well as quantitative validation based on agreement with an expert clinician's annotations of the true tumor boundaries, demonstrate our approach is highly efficient compared to traditional active contours and region growing. Due to the use of adaptable parameters in the snake evolution process, our approach outperforms the other two methods, and consistently follows an expert's annotations. Statistical tests indicated significant difference between the results produced by our approach and two other algorithms traditional snakes and region growing, while multiple comparison showed that our method consistently outperformed those algorithms, with an average overlap of 89%, over the entire data set, while traditional snakes were at 82.5% and region growing at 59.2%. Furthermore, we performed several tests that demonstrate our method's stability to different initial contours, as well as, to lower resolution images. Conclusion: Our adaptive snake algorithm can spatially adapt to diverse image characteristics, producing outlines that mimic the true tumor boundaries. Results in MR datasets are very close to an expert clinician's intuition about the tumor boundaries. [ABSTRACT FROM AUTHOR]

A fast, cost-efficient, sensitive and accurate assay method for zearalenone in edible oils is described, as an alternative to gel permeation chromatography (GPC). Oil samples were extracted with an alkaline mixture of methanol and water (methanol +10 g/l aqueous ammonium carbaminate solution, pH 9; 9 + 1, v+v). The pH of the extract was neutralized with hydrochloric acid and then concentrated to dryness. The residue was dissolved with HPLC solvent, and zearalenone was determined by high-performance liquid chromatography with fluorometric detection (HPLC-FLD). The method was successfully validated for two matrices, maize oil and rapeseed oil. The recovery rate was 87%, and the coefficient of variation was 2.8% in a rapeseed oil sample contaminated with 27 µg zearalenone/kg. At a signal-to-noise ratio of 3:1, the method detection limit was 10 µg/kg, which was considered to be adequate in view of the present European Union maximum level of 400 µg/kg. [ABSTRACT FROM AUTHOR]

Magnetic resonance imaging (MRI) presents the main diagnostic tool for differentiation and staging of renal tumors in childhood. Nephroblastoma is the most common malignant tumor in children. Radiological findings play an important role in therapy study trials of SIOP (International Society of Pediatric Oncology), especially for indicating preoperative chemotherapy. In the past few years MRI has gained great importance in imaging of nephroblastoma and has replaced computed tomography (CT). The aim of this review is to present the diagnostic possibilities of MRI in relation to the requirements of therapy studies. For nephroblastoma, MRI provides important information about tumor extent and distant metastasis. A special focus of MRI in distant staging is venous extent of the tumor into the inferior vena cava. In addition, MRI has an important role in monitoring chemotherapy and in preoperative planning by volume rendering and three-dimensional postprocessing. [ABSTRACT FROM AUTHOR]

A boy showing symptoms of a Turcot-like childhood cancer syndrome together with stigmata of neurofibromatosis type I is reported. His brother suffers from an infantile myofibromatosis, and a sister died of glioblastoma at age 7. Another 7-year-old brother is so far clinically unaffected. The parents are consanguineous. Molecular diagnosis in the index patient revealed a constitutional homozygous mutation of the mismatch repair gene PMS2. The patient was in remission of his glioblastoma (WHO grade IV) after multimodal treatment followed by retinoic acid chemoprevention for 7 years. After discontinuation of retinoic acid medication, he developed a relapse of his brain tumour together with the simultaneous occurrence of three other different HNPCC-related carcinomas. We think that retinoic acid might have provided an effective chemoprevention in this patient with homozygous mismatch repair gene defect. We propose to take a retinoic acid chemoprevention into account in children with proven biallelic PMS2 mismatch repair mutations being at highest risk concerning the development of a malignancy. [ABSTRACT FROM AUTHOR]

Wilms tumour is the most common malignant renal tumour in children. Dramatic improvements in survival have occurred as the result of advances in anaesthetic and surgical management, irradiation and chemotherapy. Current therapies are based on trials and studies primarily conducted by large multi-institutional cooperatives including the Société Internationale d’Oncologie Pédiatrique (SIOP) and the Children’s Oncology Group (COG). The primary goals are to treat patients according to well-defined risk groups in order to achieve the highest cure rates, to decrease the frequency and intensity of acute and late toxicity and to minimize the cost of therapy. The SIOP trials and studies largely focus on the issue of preoperative therapy, whereas the COG trials and studies start with primary surgery. This paper reviews prognostic factors and staging systems for Wilms tumour and its current treatment with surgery and chemotherapy. Surgery remains a crucial part of treatment for nephroblastoma, providing local primary tumour control and adequate staging and possibly controlling the metastatic spread and central vascular extension of the disease. Partial nephrectomy, when technically feasible, seems reasonable not only in those with bilateral disease but also in those with unilateral disease where the patient has urological disorders or syndromes predisposing to malignancy. Partial nephrectomy, however, is frequently not sufficient for an anaplastic variant of tumour. The late effects for Wilms tumour and its treatment are also reviewed. The treatment of Wilms tumour has been a success story, and currently in excess of 80% of children diagnosed with Wilms tumour can look forward to long-term survival, with less than 20% experiencing serious morbidity at 20 years from diagnosis. The late complications are a consequence of the type and intensity of treatment required, which in turn reflects the nature and extent of the original tumour. Continual international trial development and participation will improve matching of treatment needs with prognosis, reducing long-term complications in the majority. The advent of molecular markers of disease severity and improved functional imaging might help. [ABSTRACT FROM AUTHOR]

Unlabelled: Intensive front-line protocols have improved survival in children with malignancies; however, intensive multimodal therapy of paediatric malignancies can be associated with a significant risk of serious adverse events. Common risk scores (PRISM, PRISM III, APACHE-II) fail to predict mortality in these patients. A retrospective chart analysis of 32 paediatric cancer patients admitted to the Paediatric Intensive Care Unit (PICU) at the University Hospital of Saarland between January 2001 and December 2003 for life-threatening complications was performed. The aim of this study was to assess risk factors for short-term outcome (survival vs. non-survival when leaving the PICU) and to develop a risk score to estimate outcome in these patients. Overall survival was good (25 of 32 patients). Mortality rate was significantly related to leukaemia/lymphoma ( P = 0.029), to the number of organ failures ( P < 0.0001), neutropenia ( P = 0.001), septic shock ( P = 0.025), mechanical ventilation ( P = 0.01) and inotropic support ( P = 0.01). Employing multiple logistic regression, the strongest predictor for poor outcome was the number of organ failures ( P < 0.05). A risk score (cut-off value: >3 points for non-survival) which included the following risk factors (non-solid tumour, number of organ failures ( n > 2), neutropenia, septic shock, mechanical ventilation, and inotropic medication) yielded a sensitivity of 7/7 (95% CI: 4.56-7.00), a specificity of 23/25 (95% CI: 18.49-24.75), a positive predictive value of 23/23 (95% CI: 19.80-23.00), and a negative predictive value of 7/9 (95% CI: 3.60-8.74) for the time of admission to the PICU.Conclusion: Although our risk of mortality score is of prognostic value in assessing short-term outcome in these patients, prospective validation in a larger study cohort is mandatory. Furthermore, it must be emphasised that this risk score must not be used for decision-making in an individual patient. [ABSTRACT FROM AUTHOR]

Wilms tumor is one of the most frequent neoplasias in children. Our previous microarray screening in a large series of Wilms tumors revealed several candidate genes that are deregulated in advanced tumors and are part of the retinoic acid signaling pathway. To investigate whether retinoic acid could be employed as a novel therapeutic agent in these tumors, we treated cultured Wilms tumor cells with different concentrations of all-trans retinoic acid (ATRA) and assessed gene expression changes by real-time RT–PCR as well as microarray analysis. Several genes like RARRES1, RARRES3, CTGF, CKS2, CCNA2, IGFBP3, UBE2C, CCL2 or ITM2B that were previously found to be deregulated in advanced tumors exhibited opposite expression changes after ATRA treatment. In addition to enhanced retinoid signaling, the transforming growth factor-beta (TGFβ) pathway was strongly activated by ATRA treatment of Wilms tumor cells. Both the retinoic acid and the TGFβ pathway mediate inhibition of cell growth. These findings represent the first molecular evidence of a potential benefit from ATRA treatment in Wilms tumors.Oncogene (2005) 24, 5246–5251. doi:10.1038/sj.onc.1208725; published online 9 May 2005 [ABSTRACT FROM AUTHOR]

Nephroblastomatosis is a paediatric renal disease that may undergo malignant transformation. When neoadjuvant chemotherapy is indicated for nephroblastomatosis or bilateral Wilms' tumours, exact volumetric analysis using high-speed data processing and visualization may aid in determining tumour response. Using 3D-volume-rendering software, the 0.5-T MRI data of a 2-year-old girl with bilateral nephroblastomatosis was analysed. Exact volume determination of foci of nephroblastomatosis was performed by automatic and manual segmentation, and the relation to normal renal parenchyma was determined over a 12-month period. At the first visit, 80% (460/547 ml) of the extremely enlarged right kidney was due to nephroblastomatosis. Total tumour volume within the right kidney decreased to 74 ml under chemotherapy. Volume analysis of the two emerging right-sided masses after treatment correctly suggested Wilms' tumour. Three-dimensional rendering of the growing masses aided the surgeon in nephron-sparing surgery during tumour resection. [ABSTRACT FROM AUTHOR]

Unusual ocular symptoms observed during intravenous treatment with anti-disialoganglioside antibody (Ab) in children suffering from neuroblastoma were analyzed and the results reported. Within the framework of the German Collaborative Neuroblastoma Study NB97, 85 children with high-risk neuroblastoma received anti-GD2 monoclonal antibody ch14.18 intravenously. Side effects were regularly reported to the study center. Ocular symptoms were recorded in clinical detail, duration and development over time. Symptoms of a parasympathetic deficit corresponding to internal ophthalmoplegia, i.e. mydriasis and accommodation deficit, were found in 10 patients. They were uni- or bilateral, began after the termination of Ab infusion and improved or disappeared in all surviving children. They did not reappear or worsen upon repeated Ab infusions. The pathophysiology of these disorders remains poorly understood. It is concluded that during systemic treatment with the anti-GD2 antibody ch14.18, reversible symptoms of parasympathetic denervation of the eye may occur which, however, do not warrant termination of this treatment. [ABSTRACT FROM AUTHOR]

A 17 year old patient suffering from thalassemia, who had been dependent on transfusions since the age of three, showed an increasing incomplete sensory and motor transverse cord lesion below T-10. The causes were found to be multiple epidural erythropoietic foci, confirmed by myelography, CT, and biopsy. The lesions were located in the thoracic, lumbar, and sacral regions. Complete remission of the neurological symptoms was achieved by an immediate hypertransfusion regimen and subsequent local radiation therapy of the neuroaxis. Thirty-six cases of thalassemia and spinal space occupying lesions have been reported in the literature. The therapeutic results of these are presented and compared with our findings. [ABSTRACT FROM AUTHOR]

A case is presented of a 3-year-old boy with a mesenchymal chondrosarcoma extending from the 1st to the 5th lumbar vertebra. This is the youngest case of a mesenchymal chondrasarcoma located outside the skeleton or in the C.N.S. After assumed total excision with subsequent radiotherapy and chemotherapy, local tumor recurrence and (later) systemic metastases were detected. Standard therapy should include radical excision because of the high incidence of local recurrence and subsequent radiotherapy because of the expected high incidence of tumor cells in the CSF. The value of chemotherapy cannot be assessed, as it has been applied in only one other case found in the literature. [ABSTRACT FROM AUTHOR]

Cytogenetic analysis of a human embryonal rhabdomyosarcoma revealed a near-diploid karyotype with structural chromosome aberrations not involving the typical rearrangements of rhabdomyosarcomas, plus a large number of double minutes. Comparative genomic hybridization revealed a previously undescribed site of DNA amplification on the short arm of chromosome 1 (band 1p32-33). [ABSTRACT FROM AUTHOR]

Objectives. To describe the imaging features of nephroblastomatosis with US, CT and MR, to point out characteristics of differentiation between nephrogenic rests (NR) and Wilms' tumour (WT) and to determine the most appropriate imaging modality. Materials and methods. We reviewed the US, CT and MR images of 29 cases of histopathologically confirmed nephroblastomatosis sent to our department for reference evaluation (German nephroblastoma study). The series included 17 kidneys with NR, 6 kidneys with WT and 32 kidneys with both NR and WT. Results. NR presented as multinodular, peripheral, cortical lesions, the diffuse form of distribution being less common. Foci were homogeneous and of low echogenicity, density or signal intensity. The lesions were most clearly depicted with contrast-enhanced CT and T1-weighted (T1-W) MR images. Lesions smaller than 1 cm were rarely identified by US. The most reliable criterion to differentiate NR from WT was their homogeneity. Conclusions. Contrast-enhanced CT and T1-W MR images are of similar potential and superior to US in the diagnosis of nephroblastomatosis. Due to the significant radiation dose of serial CT, MR imaging should be the method of choice wherever it is available. The cost-effectiveness and availability of US makes it ideal for serial follow-up of known lesions. [ABSTRACT FROM AUTHOR]

The article presents a medical case regarding a 2.5-week-old boy with intermittent episodes of gross hematuria and cystic renal mass. The urine analyses of the infant revealed a mild microhematuria and the urine culture was negative. A solid lesion of the upper pole and a mild dilatation of the upper pole collecting system of the left kidney was seen through the magnetic resonance imaging.

The focuses on the diagnosis regarding the case of an infant with hematuria and cystic renal mass. The evaluation of radiological images and histopathological sections confirmed the diagnosis of ossifying renal tumor of infancy. The total nephrectomy that the patient had undergone showed no evidence of tumor recurrence or other renal disease.

Background: Congenital mesoblastic nephroma is the most common solid renal tumor in neonates. Therefore, patients <3 months of age are advised to undergo upfront nephrectomy, whereas invasive procedures at diagnosis in patients ≥3 months of age are discouraged by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Nevertheless, discriminating congenital mesoblastic nephroma, especially from the more common Wilms tumor, solely based on imaging remains difficult. Recently, magnetic resonance imaging (MRI) has become the preferred modality. Studies focusing on MRI characteristics of congenital mesoblastic nephroma are limited.This study aims to identify diagnostic MRI characteristics of congenital mesoblastic nephroma in the largest series of patients to date.In this retrospective multicenter study, five SIOP-RTSG national review radiologists identified 52 diagnostic MRIs of histologically proven congenital mesoblastic nephromas. MRI was performed following SIOP-RTSG protocols, while radiologists assessed their national cases using a validated case report form.Patients (24/52 classic, 11/52 cellular, and 15/52 mixed type congenital mesoblastic nephroma, 2/52 unknown) had a median age of 1 month (range 1 day–3 months). Classic type congenital mesoblastic nephroma appeared homogeneous with a lack of hemorrhage, necrosis and/or cysts, showing a concentric ring sign in 14 (58.3%) patients. Cellular and mixed type congenital mesoblastic nephroma appeared more heterogeneous and were larger (311.6 and 174.2 cm3, respectively, versus 41.0 cm3 for the classic type (P<0.001)). All cases were predominantly T2-weighted isointense and T1-weighted hypointense, and mean overall apparent diffusion coefficient values ranged from 1.05–1.10×10−3 mm2/s.This retrospective international collaborative study showed classic type congenital mesoblastic nephroma predominantly presented as a homogeneous T2-weighted isointense mass with a typical concentric ring sign, whereas the cellular type appeared more heterogeneous. Future studies may use identified MRI characteristic of congenital mesoblastic nephroma for validation and for exploring the discriminative non-invasive value of MRI, especially from Wilms tumor.Objective: Congenital mesoblastic nephroma is the most common solid renal tumor in neonates. Therefore, patients <3 months of age are advised to undergo upfront nephrectomy, whereas invasive procedures at diagnosis in patients ≥3 months of age are discouraged by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Nevertheless, discriminating congenital mesoblastic nephroma, especially from the more common Wilms tumor, solely based on imaging remains difficult. Recently, magnetic resonance imaging (MRI) has become the preferred modality. Studies focusing on MRI characteristics of congenital mesoblastic nephroma are limited.This study aims to identify diagnostic MRI characteristics of congenital mesoblastic nephroma in the largest series of patients to date.In this retrospective multicenter study, five SIOP-RTSG national review radiologists identified 52 diagnostic MRIs of histologically proven congenital mesoblastic nephromas. MRI was performed following SIOP-RTSG protocols, while radiologists assessed their national cases using a validated case report form.Patients (24/52 classic, 11/52 cellular, and 15/52 mixed type congenital mesoblastic nephroma, 2/52 unknown) had a median age of 1 month (range 1 day–3 months). Classic type congenital mesoblastic nephroma appeared homogeneous with a lack of hemorrhage, necrosis and/or cysts, showing a concentric ring sign in 14 (58.3%) patients. Cellular and mixed type congenital mesoblastic nephroma appeared more heterogeneous and were larger (311.6 and 174.2 cm3, respectively, versus 41.0 cm3 for the classic type (P<0.001)). All cases were predominantly T2-weighted isointense and T1-weighted hypointense, and mean overall apparent diffusion coefficient values ranged from 1.05–1.10×10−3 mm2/s.This retrospective international collaborative study showed classic type congenital mesoblastic nephroma predominantly presented as a homogeneous T2-weighted isointense mass with a typical concentric ring sign, whereas the cellular type appeared more heterogeneous. Future studies may use identified MRI characteristic of congenital mesoblastic nephroma for validation and for exploring the discriminative non-invasive value of MRI, especially from Wilms tumor.Materials and methods: Congenital mesoblastic nephroma is the most common solid renal tumor in neonates. Therefore, patients <3 months of age are advised to undergo upfront nephrectomy, whereas invasive procedures at diagnosis in patients ≥3 months of age are discouraged by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Nevertheless, discriminating congenital mesoblastic nephroma, especially from the more common Wilms tumor, solely based on imaging remains difficult. Recently, magnetic resonance imaging (MRI) has become the preferred modality. Studies focusing on MRI characteristics of congenital mesoblastic nephroma are limited.This study aims to identify diagnostic MRI characteristics of congenital mesoblastic nephroma in the largest series of patients to date.In this retrospective multicenter study, five SIOP-RTSG national review radiologists identified 52 diagnostic MRIs of histologically proven congenital mesoblastic nephromas. MRI was performed following SIOP-RTSG protocols, while radiologists assessed their national cases using a validated case report form.Patients (24/52 classic, 11/52 cellular, and 15/52 mixed type congenital mesoblastic nephroma, 2/52 unknown) had a median age of 1 month (range 1 day–3 months). Classic type congenital mesoblastic nephroma appeared homogeneous with a lack of hemorrhage, necrosis and/or cysts, showing a concentric ring sign in 14 (58.3%) patients. Cellular and mixed type congenital mesoblastic nephroma appeared more heterogeneous and were larger (311.6 and 174.2 cm3, respectively, versus 41.0 cm3 for the classic type (P<0.001)). All cases were predominantly T2-weighted isointense and T1-weighted hypointense, and mean overall apparent diffusion coefficient values ranged from 1.05–1.10×10−3 mm2/s.This retrospective international collaborative study showed classic type congenital mesoblastic nephroma predominantly presented as a homogeneous T2-weighted isointense mass with a typical concentric ring sign, whereas the cellular type appeared more heterogeneous. Future studies may use identified MRI characteristic of congenital mesoblastic nephroma for validation and for exploring the discriminative non-invasive value of MRI, especially from Wilms tumor.Results: Congenital mesoblastic nephroma is the most common solid renal tumor in neonates. Therefore, patients <3 months of age are advised to undergo upfront nephrectomy, whereas invasive procedures at diagnosis in patients ≥3 months of age are discouraged by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Nevertheless, discriminating congenital mesoblastic nephroma, especially from the more common Wilms tumor, solely based on imaging remains difficult. Recently, magnetic resonance imaging (MRI) has become the preferred modality. Studies focusing on MRI characteristics of congenital mesoblastic nephroma are limited.This study aims to identify diagnostic MRI characteristics of congenital mesoblastic nephroma in the largest series of patients to date.In this retrospective multicenter study, five SIOP-RTSG national review radiologists identified 52 diagnostic MRIs of histologically proven congenital mesoblastic nephromas. MRI was performed following SIOP-RTSG protocols, while radiologists assessed their national cases using a validated case report form.Patients (24/52 classic, 11/52 cellular, and 15/52 mixed type congenital mesoblastic nephroma, 2/52 unknown) had a median age of 1 month (range 1 day–3 months). Classic type congenital mesoblastic nephroma appeared homogeneous with a lack of hemorrhage, necrosis and/or cysts, showing a concentric ring sign in 14 (58.3%) patients. Cellular and mixed type congenital mesoblastic nephroma appeared more heterogeneous and were larger (311.6 and 174.2 cm3, respectively, versus 41.0 cm3 for the classic type (P<0.001)). All cases were predominantly T2-weighted isointense and T1-weighted hypointense, and mean overall apparent diffusion coefficient values ranged from 1.05–1.10×10−3 mm2/s.This retrospective international collaborative study showed classic type congenital mesoblastic nephroma predominantly presented as a homogeneous T2-weighted isointense mass with a typical concentric ring sign, whereas the cellular type appeared more heterogeneous. Future studies may use identified MRI characteristic of congenital mesoblastic nephroma for validation and for exploring the discriminative non-invasive value of MRI, especially from Wilms tumor.Conclusion: Congenital mesoblastic nephroma is the most common solid renal tumor in neonates. Therefore, patients <3 months of age are advised to undergo upfront nephrectomy, whereas invasive procedures at diagnosis in patients ≥3 months of age are discouraged by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Nevertheless, discriminating congenital mesoblastic nephroma, especially from the more common Wilms tumor, solely based on imaging remains difficult. Recently, magnetic resonance imaging (MRI) has become the preferred modality. Studies focusing on MRI characteristics of congenital mesoblastic nephroma are limited.This study aims to identify diagnostic MRI characteristics of congenital mesoblastic nephroma in the largest series of patients to date.In this retrospective multicenter study, five SIOP-RTSG national review radiologists identified 52 diagnostic MRIs of histologically proven congenital mesoblastic nephromas. MRI was performed following SIOP-RTSG protocols, while radiologists assessed their national cases using a validated case report form.Patients (24/52 classic, 11/52 cellular, and 15/52 mixed type congenital mesoblastic nephroma, 2/52 unknown) had a median age of 1 month (range 1 day–3 months). Classic type congenital mesoblastic nephroma appeared homogeneous with a lack of hemorrhage, necrosis and/or cysts, showing a concentric ring sign in 14 (58.3%) patients. Cellular and mixed type congenital mesoblastic nephroma appeared more heterogeneous and were larger (311.6 and 174.2 cm3, respectively, versus 41.0 cm3 for the classic type (P<0.001)). All cases were predominantly T2-weighted isointense and T1-weighted hypointense, and mean overall apparent diffusion coefficient values ranged from 1.05–1.10×10−3 mm2/s.This retrospective international collaborative study showed classic type congenital mesoblastic nephroma predominantly presented as a homogeneous T2-weighted isointense mass with a typical concentric ring sign, whereas the cellular type appeared more heterogeneous. Future studies may use identified MRI characteristic of congenital mesoblastic nephroma for validation and for exploring the discriminative non-invasive value of MRI, especially from Wilms tumor.Graphical Abstract: Congenital mesoblastic nephroma is the most common solid renal tumor in neonates. Therefore, patients <3 months of age are advised to undergo upfront nephrectomy, whereas invasive procedures at diagnosis in patients ≥3 months of age are discouraged by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Nevertheless, discriminating congenital mesoblastic nephroma, especially from the more common Wilms tumor, solely based on imaging remains difficult. Recently, magnetic resonance imaging (MRI) has become the preferred modality. Studies focusing on MRI characteristics of congenital mesoblastic nephroma are limited.This study aims to identify diagnostic MRI characteristics of congenital mesoblastic nephroma in the largest series of patients to date.In this retrospective multicenter study, five SIOP-RTSG national review radiologists identified 52 diagnostic MRIs of histologically proven congenital mesoblastic nephromas. MRI was performed following SIOP-RTSG protocols, while radiologists assessed their national cases using a validated case report form.Patients (24/52 classic, 11/52 cellular, and 15/52 mixed type congenital mesoblastic nephroma, 2/52 unknown) had a median age of 1 month (range 1 day–3 months). Classic type congenital mesoblastic nephroma appeared homogeneous with a lack of hemorrhage, necrosis and/or cysts, showing a concentric ring sign in 14 (58.3%) patients. Cellular and mixed type congenital mesoblastic nephroma appeared more heterogeneous and were larger (311.6 and 174.2 cm3, respectively, versus 41.0 cm3 for the classic type (P<0.001)). All cases were predominantly T2-weighted isointense and T1-weighted hypointense, and mean overall apparent diffusion coefficient values ranged from 1.05–1.10×10−3 mm2/s.This retrospective international collaborative study showed classic type congenital mesoblastic nephroma predominantly presented as a homogeneous T2-weighted isointense mass with a typical concentric ring sign, whereas the cellular type appeared more heterogeneous. Future studies may use identified MRI characteristic of congenital mesoblastic nephroma for validation and for exploring the discriminative non-invasive value of MRI, especially from Wilms tumor. [ABSTRACT FROM AUTHOR]

The international precision oncology program INFORM enrolls relapsed/refractory pediatric cancer patients for comprehensive molecular analysis. We report a two-year pilot study implementing ex vivo drug sensitivity profiling (DSP) using a library of 75-78 clinically relevant drugs. We included 132 viable tumor samples from 35 pediatric oncology centers in seven countries. DSP was conducted on multicellular fresh tumor tissue spheroid cultures in 384-well plates with an overall mean processing time of three weeks. In 89 cases (67%), sufficient viable tissue was received; 69 (78%) passed internal quality controls. The DSP results matched the identified molecular targets, including BRAF, ALK, MET, and TP53 status. Drug vulnerabilities were identified in 80% of cases lacking actionable (very) high-evidence molecular events, adding value to the molecular data. Striking parallels between clinical courses and the DSP results were observed in selected patients. Overall, DSP in clinical real-time is feasible in international multicenter precision oncology programs., (© 2022. The Author(s).)