Natural history of a medulloblastoma: 30months of wait and see in a child with a cerebellar incidentaloma

Introduction: With the increasing use of neuroimaging studies, the discovery of incidental neoplastic lesions is becoming more frequent. However, standard procedures are lacking, and little is known about their optimal management. Case Report: We here present the case of a boy with a cerebellar mass incidentally discovered on a CT scan performed after head trauma. In another scan performed after another incident of head trauma 14months earlier, the lesion could be seen after retrospective examination. In view of the asymptomatic clinical and stable radiological status and the presumed diagnosis of a low-grade glioma, a watch-and-wait strategy was elected. After clinical and radiological progression was observed, the tumour was resected, 2½ years after the initial imaging study. Histological evaluation revealed a WNT pathway-activated classical medulloblastoma. Discussion: To our knowledge, this is the first description of such a long natural history and pre-symptomatic period of a medulloblastoma. The long period of stability followed by a period of accelerated tumour growth is compatible with increasing biological aggressiveness, possibly related to the stepwise accumulation of genetic change

Industrial Fibre – Quality Assessment and Influence of Crop Management in Fibre Crops: a Literature Review

Aufgrund des zunehmenden Interesses verschiedener Wirtschaftszweige an Industriefasern aus Naturfaserpflanzen wird in vorliegender Untersuchung der Stand der Forschung bezüglich der Qualitätsbeschreibung und der pflanzenbaulichen Beeinflussungsmöglichkeiten bei verschiedenen Faserpflanzen für den mitteleuropäischen Raum zusammengestellt. Neben dem Faserlein, der bereits seit einigen Jahren in wissenschaftlicher Bearbeitung ist, sind andere, bislang weitgehend unerforschte Faserpflanzen wie Hanf, Fasernessel, Kenaf und Ramie im Gespräch, die gute Anbaueignung besitzen sollen. Die vorliegende Literatur in bezug auf Faserleistung und Faserqualität ist zusammengefaßt. Allerdings ist die Vergleichbarkeit der Untersuchungen eingeschränkt, da keine Versuche unter vergleichbaren Anbaubedingungen und gleicher Methodik der Ertragsfeststellung und Qualitätsanalytik vorliegen. Es werden die verschiedenen Methoden zur Untersuchung der Faserleistung und -qualität vorgestellt und die vorhandenen Ergebnisse bei den Arten diskutiert. So wurden bei Lein und Hanf sortenabhängige Qualitätsdifferenzen nachgewiesen. In der Anbautechnik wird für Faserlein mit 2000 Körnern m–2 die zehnfache Saatmenge wie für Hanf mit 200 Körnern m–2 empfohlen. Der Hanf reagiert positiv auf Erhöhung der N-Düngung, die beim Leinanbau eher zu Lager und Ertragsabfall führt. Es liegen bislang kaum aktuelle Untersuchungen zu Fasernessel, Kenaf und Ramie vor, so dass über diese Arten erst wenig Aussagen möglich sind. Der Forschungsbedarf für eine vergleichende Bewertung der Faserpflanzen wird aufgezeigt.There is a growing interest of industries in fibres from natural fibre crops. The present paper gives an overview on the status of research in different fibre crops concerning the definition of fibre quality and the possibilities to influence the fibre quality by crop management. Besides flax, which is already under scientific research since several years, there are other, so far unexplored fibre crops, such as hemp, nettle, kenaf and ramie, under discussion. These crops are also reported to have good fibre quality. The literature concerning fibre yield and fibre quality of the fibre crops is summerized. The comparison of the results is critical, because the trials were done under different growing conditions and with different methods of analysing yield and quality. The methods of analysing fibre content and fibre quality properties are presented and the achieved results are discussed. It can be shown that there are differences in fibre quality depending on the varieties for flax and hemp. The crop management such as seed density is completely different for the fibre crops. An increase of nitrogen fertilisation improves the hemp yield, but can decrease yield due to lodging of flax. There is only little research done for nettle, kenaf and ramie. The need for research to compare the different fibre crops is pointed out

Desmoplastic small round cell tumors : Multimodality treatment and new risk factors

Background To evaluate optimal therapy and potential risk factors. Methods Data of DSRCT patients Results Median age of 60 patients was 14.5 years. Male:female ratio was 4:1. Tumors were abdominal/retroperitoneal in 56/60 (93%). 6/60 (10%) presented with a localized mass, 16/60 (27%) regionally disseminated nodes, and 38/60 (63%) with extraperitoneal metastases. At diagnosis, 23/60 (38%) patients had effusions, 4/60 (7%) a thrombosis, and 37/54 (69%) elevated CRP. 40/60 (67%) patients underwent tumor resection, 21/60 (35%) macroscopically complete. 37/60 (62%) received chemotherapy according to CEVAIE (ifosfamide, vincristine, actinomycin D, carboplatin, epirubicin, etoposide), 15/60 (25%) VAIA (ifosfamide, vincristine, adriamycin, actinomycin D) and, 5/60 (8%) P6 (cyclophosphamide, doxorubicin, vincristine, ifosfamide, etoposide). Nine received high-dose chemotherapy, 6 received regional hyperthermia, and 20 received radiotherapy. Among 25 patients achieving complete remission, 18 (72%) received metronomic therapies. Three-year event-free (EFS) and overall survival (OS) were 11% (+/- 8 confidence interval [CI] 95%) and 30% (+/- 12 CI 95%), respectively, for all patients and 26.7% (+/- 18.0 CI 95%) and 56.9% (+/- 20.4 CI 95%) for 25 patients achieving remission. Extra-abdominal site, localized disease, no effusion or ascites only, absence of thrombosis, normal CRP, complete tumor resection, and chemotherapy with VAIA correlated with EFS in univariate analysis. In multivariate analysis, significant factors were no thrombosis and chemotherapy with VAIA. In patients achieving complete remission, metronomic therapy with cyclophosphamide/vinblastine correlated with prolonged time to relapse. Conclusion Pleural effusions, venous thrombosis, and CRP elevation were identified as potential risk factors. The VAIA scheme showed best outcome. Maintenance therapy should be investigated further.Peer reviewe

Influence of Harvest Date on Morphology, Yield and Quality of Different Flax and Linseed Genotypes

Die Wahl des Erntetermins ist bei Lein für die Qualität der produzierten Fasern von großer Bedeutung. Ziel vorliegender Untersuchung war es deshalb, die Zusammenhänge zwischen Abreife, Faserentwicklung und Ertrags- und Qualitätsbildung bei Lein zu klären. Dazu wurden in 2 Umwelten Versuche mit 9 Leingenotypen durchgeführt. Es wurden die Pflanzenentwicklung beobachtet und zu verschiedenen Terminen ab der Blüte Pflanzenproben auf Leistungs- und Qualitätsmerkmale untersucht. Zusätzlich ist mittels bildanalytischer Auswertung mikroskopischer Stängelquerschnitte die Entwicklung der Einzelfasern und Faserbündel analysiert worden.Der Trockenmasseanteil des Strohs nimmt bis zur Kapselreife auf maximal 50% zu. Der Faseranteil steigt bis zur Strohreife, die relative Wasserdampfaufnahme der Fasern sinkt bis zur Kapselreife kontinuierlich ab. Mit zunehmender Abreife ist zunächst ein Anstieg der Zellzahl, später nur noch eine Vergrößerung der Zellen und Bündel festzustellen. Die Lignifizierung der Zellen und die Zellwanddicke nehmen zu, der Lumenanteil ab. Die Bedeutung des Erntetermins für die mechanischen Eigenschaften ist umwelt- und sortenabhängig. Da keine eindeutige Verschlechterung der Faserqualität zur Kapselreife nachgewiesen werden konnte, ist eine Ernte zur Kapselreife ohne Qualitätsverlust bei den Fasern möglich, wodurch eine gleichzeitige Samennutzung möglich wird.The choice of the harvest date of flax is of great importance for the quality of the produced fibres. This study was conducted to determine the correlations between ripening, fibre development and yield and quality establishment in flax. Field trials with 9 genotypes of flax and linseed were conducted in two environments. The development of the plants was investigated and plant samples were taken at different stages for the investigation of yield and quality. Additionally the development of fibre cells and fibre bundles were analysed by microscopy and image analysis of cross sections from the stems. The percentage of dry matter in the stems reaches a maximum of 50% at capsule maturity. The fibre content increases until the straw ripeness, the water absorption decreases continuous between flowering and capsule maturity. The morphological changes during the maturation are characterized first by an increase of the number of fibre cells, from straw ripening on only the size of cells and bundles enlarges. The lignification of the cells and the thickness of the cell walls rises between straw and capsule ripening, the lumen content declines. There are interactions between harvest date, environment and variety for the mechanical properties of the fibre bundles. Because there were no lower values for the fibre quality observed at capsule maturity than at straw maturity it can be concluded, that fibre flax for industrial use can be harvested at capsule maturity without loss of quality. Besides a use of the seeds from the capsules will be possible

E 4 Transfer (E=P, As) to Ni Complexes

The use of [Cp′′2Zr(η1:1-E4)] (E=P (1 a), As (1 b), Cp′′=1,3-di-tert-butyl-cyclopentadienyl) as phosphorus or arsenic source, respectively, gives access to novel stable polypnictogen transition metal complexes at ambient temperatures. The reaction of 1 a/1 b with [CpRNiBr]2 (CpR=CpBn (1,2,3,4,5-pentabenzyl-cyclopentadienyl), Cp′′′ (1,2,4-tri-tert-butyl-cyclopentadienyl)) was studied, to yield novel complexes depending on steric effects and stoichiometric ratios. Besides the transfer of the complete En unit, a degradation as well as aggregation can be observed. Thus, the prismane derivatives [(Cp′′′Ni)2(μ,η3:3-E4)] (2 a (E=P); 2 b (E=As)) or the arsenic containing cubane [(Cp′′′Ni)3(μ3-As)(As4)] (5) are formed. Furthermore, the bromine bridged cubanes of the type [(CpRNi)3{Ni(μ-Br)}(μ3-E)4]2 (CpR=Cp′′′: 6 a (E=P), 6 b (E=As), CpR=CpBn: 8 a (E=P), 8 b (E=As)) can be isolated. Here, a stepwise transfer of En units is possible, with a cyclo-E42− ligand being introduced and unprecedented triple-decker compounds of the type [{(CpRNi)3Ni(μ3-E)4}2(μ,η4:4-E′4)] (CpR=CpBn, Cp′′′; E/E′=P, As) are obtained

Elimusertib has anti-tumor activity in preclinical patient-derived pediatric solid tumor models

The small molecule inhibitor of ataxia telangiectasia and Rad3-related protein (ATR), elimusertib, is currently being tested clinically in various cancer entities in adults and children. Its preclinical anti-tumor activity in pediatric malignancies, however, is largely unknown. We here assessed the preclinical activity of elimusertib in 38 cell lines and 32 patient-derived xenograft (PDX) models derived from common pediatric solid tumor entities. Detailed in vitro and in vivo molecular characterization of the treated models enabled the evaluation of response biomarkers. Pronounced objective response rates were observed for elimusertib monotherapy in PDX, when treated with a regimen currently used in clinical trials. Strikingly, elimusertib showed stronger anti-tumor effects than some standard of care chemotherapies, particularly in alveolar rhabdomysarcoma PDX. Thus, elimusertib has strong preclinical anti-tumor activity in pediatric solid tumor models, which may translate to clinically meaningful responses in patients

Pre-operative radiotherapy is associated with superior local relapse-free survival in advanced synovial sarcoma

PURPOSE: Optimization of local therapies in synovial sarcoma (SS) considered unresectable at diagnosis is needed. We evaluated the effects of neoadjuvant versus adjuvant radiation versus surgery only on long-term outcomes. METHODS: Patients with macroscopic SS tumors before chemotherapy (IRS-group-III) in the trials CWS-81, CWS-86, CWS-91, CWS-96, CWS-2002-P and SoTiSaR-registry were analyzed. Local therapies were scheduled after 3 neoadjuvant chemotherapy cycles. RESULTS: Median age of 145 patients was 14.5 years. 106 survivors had median follow-up of 7.0 years. Tumor site was 96 extremities, 19 head-neck, 16 shoulder/hip, 14 trunk. Tumors were  10 cm in 34 patients. In a secondary resection during chemotherapy, R0-status was accomplished in 82, R1 in 30, R2 in 21 (12 missing). Radiotherapy was administered to 115 (R0 61, R1 29, R2 20, missing 5), thereof 57 before and 52 after tumor resection. 23 were treated with surgery only. For all patients, 5 year event-free (EFS) and overall survival (OS) was 68.9% ± 7.6 (95%CI) and 79.1% ± 6.9. To establish independent significance, tumor site, size, surgical results and sequencing of local therapies were analyzed in a Cox regression analysis. Variables associated with EFS and OS are site, size and sequencing of local therapies. Variables associated with local recurrence are site, surgical results and sequencing of local therapies. The only variable associated with suffering metastatic recurrence is tumor size. CONCLUSION: Differences in sequencing of local therapy procedures are independently associated with outcomes. Best local control is achieved when tumors are irradiated pre-operatively and undergo R0 or R1 resection thereafter

The German National Pandemic Cohort Network (NAPKON): rationale, study design and baseline characteristics

Schons M, Pilgram L, Reese J-P, et al. The German National Pandemic Cohort Network (NAPKON): rationale, study design and baseline characteristics. European Journal of Epidemiology . 2022.The German government initiated the Network University Medicine (NUM) in early 2020 to improve national research activities on the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic. To this end, 36 German Academic Medical Centers started to collaborate on 13 projects, with the largest being the National Pandemic Cohort Network (NAPKON). The NAPKON's goal is creating the most comprehensive Coronavirus Disease 2019 (COVID-19) cohort in Germany. Within NAPKON, adult and pediatric patients are observed in three complementary cohort platforms (Cross-Sectoral, High-Resolution and Population-Based) from the initial infection until up to three years of follow-up. Study procedures comprise comprehensive clinical and imaging diagnostics, quality-of-life assessment, patient-reported outcomes and biosampling. The three cohort platforms build on four infrastructure core units (Interaction, Biosampling, Epidemiology, and Integration) and collaborations with NUM projects. Key components of the data capture, regulatory, and data privacy are based on the German Centre for Cardiovascular Research. By April 01, 2022, 34 university and 40 non-university hospitals have enrolled 5298 patients with local data quality reviews performed on 4727 (89%). 47% were female, the median age was 52 (IQR 36-62-) and 50 pediatric cases were included. 44% of patients were hospitalized, 15% admitted to an intensive care unit, and 12% of patients deceased while enrolled. 8845 visits with biosampling in 4349 patients were conducted by April 03, 2022. In this overview article, we summarize NAPKON's design, relevant milestones including first study population characteristics, and outline the potential of NAPKON for German and international research activities.Trial registration https://clinicaltrials.gov/ct2/show/NCT04768998 . https://clinicaltrials.gov/ct2/show/NCT04747366 . https://clinicaltrials.gov/ct2/show/NCT04679584. © 2022. The Author(s)