1,198 research outputs found
Statistics of dark matter haloes expected from weak lensing surveys
The distortion of the images of faint high-redshift galaxies can be used to
probe the intervening mass distribution. This weak gravitational lensing effect
has been used recently to study the (projected) mass distribution of several
clusters at intermediate and high redshifts. In addition, the weak lensing
effect can be employed to detect (dark) matter concentrations in the Universe,
based on their mass properties alone. Thus it is feasible to obtain a
mass-selected sample of `clusters', and thereby probe the full range of their
mass-to-light ratios. We study the expected number density of such haloes which
can be detected in ongoing and future deep wide-field imaging surveys, using
the number density of haloes as predicted by the Press-Schechter theory, and
modeling their mass profile by the `universal' density profile found by
Navarro, Frenk & White. We find that in all cosmological models considered, the
number density of haloes with a signal-to-noise ratio larger than 5 exceeds 10
per square degree. With the planned MEGACAM imaging survey of ,
it will be easily possible to distinguish between the most commonly discussed
cosmological parameter sets.Comment: 8 pages, 7 figures, submitted to MNRA
Interprofessional Inconsistencies in the Diagnosis of Shoulder Instability: Survey Results of Physicians and Rehabilitation Providers
Background: Clinicians of many specialties within sports medicine care for athletes with shoulder instability, but successful outcomes are inconsistent. Consistency across specialties in the diagnosis of shoulder instability is critical for care of the athlete, yet the extent of divergence in its diagnosis is unknown.
Hypothesis: Physicians differ from rehabilitation providers in which findings they deem clinically important to differentiate shoulder instability from impingement, and in how they diagnose athlete scenarios with atraumatic shoulder instability.
Study Design: Cross-sectional study.
Methods: Physicians (orthopaedic surgeons, primary care sports medicine physicians) and rehabilitation providers (physical therapists, athletic trainers) were asked via an online survey to rate clinical factors used to diagnose shoulder instability. Clinicians were also asked to diagnose two athlete scenarios with concurrent clinical findings of atraumatic shoulder instability and impingement, differentiated by the absence or presence of a positive sulcus sign.
Results: Responses were recorded from 888 clinicians. Orthopaedic surgeons (N=170) and primary care sports medicine physicians (N=108) ranked physical examination factors as more important for the diagnosis of shoulder instability than patient history factors, whereas physical therapists (N=379) and athletic trainers (N=231) preferred patient history factors. Orthopaedic surgeons differed from physical therapists and athletic trainers in their clinical diagnoses for both scenarios (Pâ€0.001).
Conclusion: A lack of consistency exists among sports medicine clinicians in recognizing which clinical factors are important when used to diagnose shoulder instability and in diagnoses given with concurrent findings of impingement.
Level of Evidence: Level 3
Lymph node metastases are more frequent in paediatric appendiceal NET â„1.5âŻcm but without impact on outcome â data from the German MET studies
Background
Paediatric appendiceal neuroendocrine tumours (appNET) are very rare tumours, mostly detected incidentally by histopathological evaluation after appendectomy. Treatment recommendations are based on adult data considering high-risk NET as defined by European Neuroendocrine Tumour Society (ENETS) guidelines for completion right-sided hemicolectomy (RHC). Recent data suggest that less aggressive therapy may be justified.
Procedure
Analysis of children and adolescents with appNET prospectively registered with the German Malignant Endocrine Tumour (MET) studies between 1997 and 2022.
Results
By December 2022, 662 patients (64.7% females, 35.3% male) had been reported. Median age was 13.3 years [4.5â17.9], median duration of follow-up 2.2 years [0â10.9]. No distant metastases were reported. Tumour size was 2âŻcm in 3.2% of patients. WHO grade 1 and 2 tumours were diagnosed in 76.9% and 23.1% of patients, respectively. Lymphovascular invasion and lymph node metastases were associated with tumour size â„1.5âŻcm. 27.0% of patients presented with high-risk NET according to ENETS criteria. Of those, only 55.9% underwent secondary oncological right hemicolectomy. Neither distant metastases, nor recurrences or disease-related deaths occurred in patients with appendectomy only as well as in patients with completion RHC. Overall and event-free survival were both 100%.
Conclusions
Internationally harmonized consensus recommendations on treatment of children and adolescents with appendiceal NET are urgently needed to avoid completion RHC in high-risk patients
Recommendations for the Transition to Open Access in Austria
Based on 16 recommendations, efforts should be made to achieve the following goal: By 2025, a large part of all scholarly publication activity in Austria should be Open Access. In other words, the final versions of most scholarly publications (in particular all refereed journal articles and conference proceedings) resulting from the support of public resources must be freely accessible on the Internet without delay (Gold Open Access). This goal should be pursued by taking into account the different disciplinary practices and under consideration of the different disciplinary priorisations of Open Access. The resources required to meet this obligation shall be provided to the authors, or the cost of the publication venues shall be borne directly by the research organisations. The necessary funding must be brought in line with the overall funding priorities for research
Locally advanced adrenocortical carcinoma in children and adolescents â enigmatic and challenging cases
Background: Locally advanced tumors account for approximately 50% of children and adolescents with adrenocortical carcinoma (ACC), and of these, up to 50% relapse. We explored the five-item microscopic score and the pS-GRAS score for guiding management. Methods: Data from children and adolescents with COG stage II and III ACC registered in the MET studies were included. The five-item and pS-GRAS score were retrospectively calculated. Results: By December 2021, 55 patients with stage II and III (stage II n = 18, stage III n = 37) had been reported. Median age was 4.3 years [0.1â17.8], median duration of follow-up 6.0 years [0â16.7]. 3-year event-free survival (EFS) rate was 76.5% and 49.8% (p = 0.088), respectively. In stage II tumors, neither the five-item score (p = 0.872) nor pS-GRAS grouping (p = 0.218) had any effect as prognostic factors. In stage III patients, EFS was impaired in tumors with unfavorable histology according to the five-item score (100% vs. 30.8%, p = 0.018). No difference was observed for pS-GRAS groups (p = 0.798). Conclusions: In patients with COG stage III, but not stage II, the five-item score affected EFS. Further studies are needed to identify patients at risk in COG stage II
Adrenocortical tumors and pheochromocytoma/paraganglioma initially mistaken as neuroblastoma â experiences from the GPOH-MET registry
In children and adolescents, neuroblastoma (NBL), pheochromocytoma (PCC), and adrenocortical tumors (ACT) can arise from the adrenal gland. It may be difficult to distinguish between these three entities including associated extra-adrenal tumors (paraganglioma, PGL). Precise discrimination, however, is of crucial importance for management. Biopsy in ACT or PCC is potentially harmful and should be avoided whenever possible. We herein report data on 10 children and adolescents with ACT and five with PCC/PGL, previously mistaken as NBL. Two patients with adrenocortical carcinoma died due to disease progression. Two (2/9, missing data in one patient) patients with a final diagnosis of ACT clearly presented with obvious clinical signs and symptoms of steroid hormone excess, while seven patients did not. Blood analyses indicated increased levels of steroid hormones in one additional patient; however, urinary steroid metabolome analysis was not performed in any patient. Two (2/10) patients underwent tumor biopsy, and in two others tumor rupture occurred intraoperatively. In 6/10 patients, ACT diagnosis was only established by a reference pediatric pathology laboratory. Four (4/5) patients with a final diagnosis of PCC/PGL presented with clinical signs and symptoms of catecholamine excess. Urine tests indicated possible catecholamine excess in two patients, while no testing was carried out in three patients. Measurements of plasma metanephrines were not performed in any patient. None of the five patients with PCC/PGL received adrenergic blockers before surgery. In four patients, PCC/PGL diagnosis was established by a local pathologist, and in one patient diagnosis was revised to PGL by a pediatric reference pathologist. Genetic testing, performed in three out of five patients with PCC/PGL, indicated pathogenic variants of PCC/PGL susceptibility genes. The differential diagnosis of adrenal neoplasias and associated extra-adrenal tumors in children and adolescents may be challenging, necessitating interdisciplinary and multidisciplinary efforts. In ambiguous and/or hormonally inactive cases through comprehensive biochemical testing, microscopical complete tumor resection by an experienced surgeon is vital to preventing poor outcome in children and adolescents with ACT and/or PCC/PGL. Finally, specimens need to be assessed by an experienced pediatric pathologist to establish diagnosis
Outcome for pediatric adreno-cortical tumors is best predicted by the COG stage and five-item microscopic score â report from the German MET studies
Background: Adrenocortical tumors (ACTs) encompassing the adrenocortical adenoma (ACA), carcinoma (ACC), and tumors of undetermined malignant potential (ACx) are rare endocrine neoplasms with a poor prognosis. We report on pediatric ACT patients registered with the Malignant Endocrine Tumor studies and explore the EXPeRT recommendations for management. Patients: Data from the ACT patients (<18 years) were analyzed. For the risk prediction, the patients were retrospectively assigned to the COG stages and the five-item score. Results: By December 2021, 161 patients with ACT (ACA n = 51, ACx n = 19, and ACC n = 91) had been reported (the median age at the diagnosis was 4.3 years with a range of 0.1â17.8), with lymph node and distant metastases in 10.7% and 18.9% of the patients with ACC/ACx. The mean follow-up was 4.5 years (with a range of 0â16.7). The three-year overall (OS) and event-free survival (EFS) rates were 65.5% and 50.6%. In the univariate analyses, the OS was impaired for patients aged â„ 4 years (p = 0.001) with the initial biopsy (p = 0.016), tumor spillage (p = 0.028), incomplete tumor resection (p < 0.001), unfavorable histology (p = 0.047), and COG stages III/IV (p = 0.002). Multivariate analysis revealed COG stages III/IV and an unfavorable five-item score as independent negative prognostic factors for the EFS and OS. Conclusions: Age defines the clinical presentation and prognosis in pediatric ACTs. The outcome is best predicted by the COG stage and five-item score
Refractory and relapsed paediatric ACC in the MET studies â a challenging situation necessitating novel diagnostic and therapeutic concepts
Background
Paediatric adrenocortical carcinomas (ACC) are highly aggressive malignancies with a dismal prognosis in advanced and metastatic disease. Little is known about outcome of patients with refractory and relapsed (r/r) disease.
Procedure
National retrospective multicentre study including r/r ACC diagnosed in patients aged <18 years registered in the MET studies between January 1997 and December 2021
Results
A total of 16 patients (5 male; median age 12.9 years) with refractory disease were included. Median time to progression was 0.6 years [0.0-1.3]. Site of progression was locoregional (n=1), distant (n=3), and combined (n=12). 3-year overall (OS) and progression-free (PFS) survival were both 0%.
Thirty patients with relapse (11 male; median age 7.3 years) were identified. Median time to relapse was 0.7 years [0.1-3.2]. Site of relapse was locoregional (n=8), distant (n=15), and combined (n=7). At last follow-up, 20 patients had died of disease or complications or were alive with disease, 10 patients were in second complete remission (median follow-up: 6.8 years [0-10.5]). 3-year OS and PFS following relapse were 39.1% and 31.9%. Survival was superior in patients with distant relapse (59.6%) compared to locoregional (28.6%) and combined (14.3%) (p=0.028) and in patients with complete surgical resection of all sites of recurrence (70.0%) compared to incomplete (21.4%) and no surgery (0%) (p=0.003).
Conclusions
For patients nonresponsive to first-line therapy or who experience relapse, prognosis is dismal and options are scarce. Site of relapse and resectability define prognosis. Novel therapeutic concepts are needed to improve the outcome of paediatric patients with r/r ACC
Second-line treatment of pediatric patients with relapsed rhabdomyosarcoma adapted to initial risk stratification: Data of the European Soft Tissue Sarcoma Registry (SoTiSaR).
BACKGROUND
Outcome of relapsed disease of localized rhabdomyosarcoma remains poor. An individual treatment approach considering the initial systemic treatment and risk group was included in the Cooperative Weichteilsarkom Studiengruppe (CWS) Guidance.
METHODS
Second-line chemotherapy (sCHT) ACCTTIVE based on anthracyclines (adriamycin, carboplatin, cyclophosphamide, topotecan, vincristine, etoposide) was recommended for patients with initial low- (LR), standard- (SR), and high-risk (HR) group after initial treatment without anthracyclines. TECC (topotecan, etoposide, carboplatin, cyclophosphamide) was recommended after initial anthracycline-based regimen in the very high-risk (VHR) group. Data of patients with relapse (n = 68) registered in the European Soft Tissue Sarcoma Registry SoTiSaR (2009-2018) were retrospectively analyzed.
RESULTS
Patients of initial LR (n = 2), SR (n = 16), HR (n = 41), and VHR (n = 9) group relapsed. sCHT consisted of ACCTTIVE (n = 36), TECC (n = 12), or other (n = 15). Resection was performed in 40/68 (59%) patients and/or radiotherapy in 47/68 (69%). Initial risk stratification, pattern/time to relapse, and achievement of second complete remission were significant prognostic factors. Microscopically incomplete resection with additional radiotherapy was not inferior to microscopically complete resection (p = .17). The 5-year event-free survival (EFS) and overall survival (OS) were 26% (±12%) and 31% (±14%). The 5-year OS of patients with relapse of SR, HR, and VHR groups was 80% (±21%), 20% (±16%), and 13% (±23%, p = .008), respectively.
CONCLUSION
Adapted systemic treatment of relapsed disease considering the initial risk group and initial treatment is reasonable. New treatment options are needed for patients of initial HR and VHR groups
Serum and Glucocorticoid Inducible Kinase 1-Sensitive Survival, Proliferation and Migration of Rhabdomyosarcoma Cells
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