Extracranial Germ Cell Tumors in Children: Ten Years of Experience in Three Children’s Medical Centers in Shanghai
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ABSTRACT:
SUBMITTER: Jiang S
PROVIDER: S-EPMC10670163 | biostudies-literature | 2023 Nov
REPOSITORIES: biostudies-literature
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Project description:BackgroundGerm cell tumors (GCTs) comprise a rare and heterogeneous group of neoplasms presenting different clinical and histological characteristics, leading to a challenging scenario in clinical practice. Diffusion-weighted imaging (DWI) has been suggested as an indirect marker of tumor density and cellularity and could be used to monitor therapeutic response. However, its role in pediatric GCTs needs to be clarified.PurposeHere, we evaluated the features of DWI in pediatric extracranial GCTs in a reference Brazilian institution.Material and methodsWe included 43 pediatric patients with primary GCTs treated between 2008 and 2022 in Hospital de Amor de Barretos. The patients' MRI images included T1-weighted without contrast, T2-weighted, DWI and apparent diffusion coefficient (ADC) maps. DWI was evaluated in the section that exhibited the greatest restricted diffusion in the largest hypersignal area of the image. The lowest ADC value was determined to define the region of interest (ROI). We used a small ROI, avoiding necrotic, adipose tissue, noisy or nonenhancing lesion voxels as recommended. ROI determination was established by visual inspection by two radiologists in accordance. We used two values of b (b = 50 mm2/s or b = 800) for ADC values.ResultsThe highest mean ADC (mADC) value was observed in pure teratomas (1,403.50 ± 161.76 x10-3 mm2/s; mean ± SD) compared to other histologies (yolk sac, mixed teratoma, dysgerminoma and mixed GCT) of GCT (p<0.001). Furthermore, ROC analysis determined a cutoff mADC value of 1,179.00 x 10-3 mm2/s that differentiated pure teratomas from the other GCT histologies with a sensitivity of 95.8% and a specificity of 92.9% (AUC = 0.979; p<0.01). A significant increase in mADC was observed for malignant GCTs in treatment (1,197.00 ± 372.00 mm2/s; p<0.001) compared to that exhibited at the time of diagnosis (780.00 ± 168.00 mm2/s; mean ± SD. Our findings suggest that mADC assessment could be used as a tool to distinguish pure teratomas from malignant CGT histologies at diagnosis. Additionally, we demonstrated reasonable evidence that it could be used as a complementary tool to monitor treatment response in patients with malignant GCT.
| S-EPMC10688858 | biostudies-literature
Project description:UNLABELLED: The advantages of sonographic examination are well known, but its main disadvantage is that it might lead to overdiagnosis, which might cause overtreatment. Variations in the incidence of developmental dysplasia of the hip are well known. We ascertained the incidence of neonatal sonographic developmental dysplasia of the hip without considering the development of those joints during followup. All 45,497 neonates (90,994 hips) born in our institute between January 1992 and December 2001 were examined clinically and sonographically during the first 48 hours of life. Sonography was performed according to Graf's method, which considers mild hip sonographic abnormalities as Type IIa. We evaluated the different severity type incidence pattern and its influence on the total incidence during and between the investigated years. According to our study, sonographic Type IIa has major effects on the incidence of overall developmental dysplasia of the hip with a correlation coefficient of 0.95, whereas more severe sonographic abnormalities show relatively stable incidence patterns. LEVEL OF EVIDENCE: Level I, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.
| S-EPMC2504674 | biostudies-other
Project description:Although pediatric malignant extracranial germ-cell tumors (meGCTs) are among the most chemosensitive solid tumors, a group of patients relapse and die of disease. To identify new markers predicting clinical outcome, we examined the prognostic relevance of tumor-infiltrating T lymphocytes (TILs) and the expression of PD-1 and PD-L1 in a cohort of pediatric meGCTs by in situ immunohistochemistry. MeGCTs were variously infiltrated by T cell-subtypes according to the tumor subtype, tumor location and age at diagnosis. We distinguished three different phenotypes: i) tumors not infiltrated by T cells (immature teratomas and half of the yolk sac tumors), ii) tumors highly infiltrated by CD8+ T cells expressing PD-1, which identifies activated tumor-reactive T cells (seminomas and dysgerminomas), iii) tumors highly infiltrated by CD8+ T cells within an immunosuppressive tumor microenvironment characterized by CD4+FOXP3+ Treg cells and PD-L1-expressing tumor cells (embryonal carcinomas, choriocarcinomas and the remaining yolk sac tumors). Tumor subtypes belonging mixed meGCTs were variously infiltrated, suggesting the coexistence of multiple immune microenvironments either facilitating or precluding the entry of T cells. These findings support the hypothesis that TILs influence the development of meGCTs and might be of clinical relevance to improve risk stratification and the treatment of pediatric patients.
| S-EPMC6343784 | biostudies-literature
Project description:Introduction: Patient surveys are an established tool for quality control in healthcare organizations. This report looks at the design and development of the annual patient surveys carried out among breast cancer patients treated in the Breast Centers of North Rhine-Westphalia and discusses selected findings from 10 consecutive years. Material and Methods: Since 2006 the Institute for Medical Sociology, Health Services Research and Rehabilitation Science (IMVR) of the University of Cologne has carried out an annual survey of breast cancer patients using the Cologne Patient Questionnaire for Breast Cancer. The patients included in the survey have been diagnosed with primary breast cancer and undergo surgery between February and July in one of the Breast Centers in North Rhine-Westphalia accredited by the medical association of Westphalia-Lippe. The questionnaire and the type of feedback given to the Breast Centers were comprehensively revised in 2014. Selected results collected over the survey period were analyzed descriptively. Results: The survey period of 10 years provides information on the actual medical care delivered in the Breast Centers based on the data obtained in this period from around 40 000 patients. Some areas showed positive developments over time. Conclusion: The approach used to survey patients, the Cologne Patient Questionnaire for Breast Cancer 2.0 and the benchmark-focused feedback provide an impetus for organizational learning in Breast Centers. The concept could also be used in other healthcare organizations to stimulate learning and improve healthcare services.
| S-EPMC4739718 | biostudies-literature
Project description:Mesenteric Panniculitis (MP) is predominately a disease of the small bowel of unknown etiology. Characterized by Fibrosis and chronic inflammation of fatty tissue of the mesentery in the small bowel. It is commonly diagnosed based on computed tomography (CT scan) with IV contrast and biopsies in equivocal cases. We conducted a retrospective study from 2011 to 2020. We analyzed the medical records of 40 patients with Mesenteric Panniculitis. The most common presentation was vague abdominal symptoms. We successfully managed the patients medically with prednisone, azathioprine, colchicine, or a combination. Patients on prednisolone showed good responses clinically and radiologically during follow-up. One patient was operated on and didn't respond to medical therapy.
| S-EPMC9422171 | biostudies-literature
Project description:Precision medicine utilizing the genetic information of genes involved in the metabolism and disposition of drugs can not only improve drug efficacy but also prevent or minimize adverse events. Polypharmacy is common among multimorbid patients and is associated with increased adverse events. One of the main objectives in health care is safe and efficacious drug therapy, which is directly correlated to the individual response to treatment. Precision medicine can increase drug safety in many scenarios, including polypharmacy. In this report, we share our experience utilizing precision medicine over the past ten years. Based on our experience using pharmacogenetic (PGx)-informed prescribing, we implemented a five-step precision medicine protocol (5SPM) that includes the assessment of the biological-clinical characteristics of the patient, current and past prescription history, and the patient's PGx test results. To illustrate our approach, we present cases highlighting the clinical relevance of precision medicine with a focus on patients with a complex history and polypharmacy.
| S-EPMC8779486 | biostudies-literature
Project description:BackgroundSpecialized adult care of phenylketonuria (PKU) patients is of increasing importance. Adult outpatient clinics for inherited errors of metabolism can help to achieve this task, but experience is limited. Ten years after establishment of a coordinated transition process and specialised adult care for inherited metabolic diseases, adult PKU care was evaluated with respect to metabolic control, therapy satisfaction, life satisfaction, sociodemographic data, economical welfare as well as pregnancy outcome.MethodsAll PKU patients transferred from paediatric to adult care between 2005 and 2015 were identified. A retrospective data analysis and a cross-sectional survey in a sub-cohort of 30 patients including a questionnaire for assessing quality of life (FLZm) were performed as a single-centre investigation at the metabolic department of the University Hospital Leipzig, Germany. For statistical analysis, Mann-Whitney-U-test, t-test for independent samples, ANOVA and chi square test were used as appropriate.Results96 PKU patients (56 females/40 males; median age 32 years, range 18-62) were included. In the last 3-year period, 81% of the transferred patients still kept contact to the adult care centre. Metabolic control was stable over the evaluation period and dried blood phenylalanine concentrations mostly remained within the therapeutic range (median 673.0 μmol/l, range 213.0-1381.1). Sociodemographic data, economical welfare and life satisfaction data were comparable to data from the general population. However, differences could be revealed when splitting the cohort according to time of diagnosis and to management during childhood. 83% of the PKU adults were satisfied with the transition process and current adult care. 25 completed pregnancies were supervised; three newborns, born after unplanned pregnancy, showed characteristic symptoms of maternal PKU syndrome.ConclusionsContinuous care for adult PKU patients in a specialized outpatient clinic is successful, leading to good to satisfactory metabolic control and social outcomes. Uninterrupted good metabolic treatment throughout childhood and adolescence positively influences educational, professional and economic success in later life. Further effort in specialized paediatric and adult metabolic care is needed to prevent loss of follow-up and to support the recommended life-long treatment and/or care.
| S-EPMC4806494 | biostudies-literature
Project description:Bioprosthetic valves for tricuspid valve replacement (TVR) have become increasingly popular in recent years, but mechanical valves remain valuable, particularly for the patients who want to avoid reoperation for bioprostheses malfunction. The aim of this study was to review our 10-year experience in adult patients who underwent TVR with the St. Jude Medical (SJM) valve. From 2005 to 2015, 265 TVRs with SJM valves were performed at our institution. The mean age at operation was 44.1 ± 9.7 years, and 207 cases (78.1%) were female. The mean follow-up was 4.9 ± 2.7 years. Preoperative atrial fibrillation was present in 199 cases (75.1%) and ascites in 26 (9.8%). Of all cases, 88.7% were characterized as New York Heart Association class III or IV. The hospital mortality was 6.4%. There were 9 deaths (3.8%) during late follow-up. The overall survival rates were 89.2% ± 2.2% at 5 years and 86.6% ± 2.9% at 10 years. The linearized rates of valve thrombosis and bleeding events were 0.8%/patient-year and 1.5%/patient-year, respectively. Three cases (1.3%) were reoperated due to prosthetic valve thrombosis. There was no reoperation for sperivalvular leakage and structural failure. The freedom from reoperation was 98.6% ± 0.8% at 5 years and 98.6% ± 0.8% at 10 years. The SJM valve in the tricuspid position is a reliable mechanical prosthesis with a low rate of valve thrombosis and reoperation. It is a reasonable choice for the patients who require mechanical valve replacement in the tricuspid position.
| S-EPMC6226498 | biostudies-literature
Project description:The People's Republic of Bangladesh has been working to eliminate visceral leishmaniasis or Kala-azar cases since there was a memorandum of understanding signed between neighboring countries in 2005. As a part of the elimination activity, 44 cases of Kala-azar were diagnosed and treated in the regional referral center Dhaka Medical College Hospital (DMCH) during the last three years, which is reported here. Confirmed leishmaniasis cases were included. Patients attending this specialized center with different demographic characteristics and varied presentations with laboratory findings were reviewed and recorded in a structured case record form. Ethical clearance was obtained prior to starting the study. A total of 44 patients with leishmaniasis were reviewed. Approximately 89% (n = 39) were New Kala-azar (NKA), 7% (n = 3) were Relapse Kala-azar (Relapse KA), only one case (2%) was Kala-azar Treatment Failure (KATF) and Post Kala-azar Dermal Leishmaniasis (PKDL) for both. The mean age of presentation was 32 years. Forty percent of patients had houses made by mud, 26% by tin shed, and the rest lived in buildings and semi-buildings. The predominant clinical features were fever (90.9%), pallor (88.6%), splenomegaly (81.8%) and hepatomegaly (68.2%). rK39 was positive in 90.7% of cases, and 94.4% of cases were positive for LD bodies on splenic aspirate. Of all, 90.90% were treated with Inj. Liposomal amphotericin B and 9.10% with the combination of Inj. Liposomal Amphotericin B and Inj. Miltefosine. Moving forward to the elimination of leishmaniasis from Bangladesh, the study highlights the status, characteristics and treatment of the disease in the country.
| S-EPMC7679253 | biostudies-literature
Project description:BACKGROUND: Barrett's metaplasia is a premalignant condition for which screening is recommended. AIMS/METHODS: Annual screening endoscopies have been performed on all subjects suitable for surgery for many years. To examine the benefits to patients of continuing this policy 10 years' experience of screening for Barrett's oesophagus was reviewed. RESULTS: Of the 29,374 endoscopies performed over this period, Barrett's metaplasia was present in 1.4%. There was an approximate equal sex distribution with the majority of cases being identified in patients over 50 years old. Thirty five per cent of these patients were suitable to enter a yearly screening programme. During screening, the length of oesophagus involved did not vary over time. As a result of 379 endoscopies performed for screening purposes, only one patient was identified as developing carcinoma solely as a result of the screening programme. Two further patients had carcinoma identified as a result of progressive dysphagia requiring additional endoscopies. All three patients who developed carcinoma while in the screening programme had "long segment" (> 8 cm) regions of Barrett's metaplasia with a stricture present at the initial endoscopy. CONCLUSIONS: The poor yield from this screening programme suggests that the policy should be reconsidered. Resources may be better spent on focusing on patients who present with ulceration or stricture in addition to Barrett's metaplasia.
| S-EPMC1891490 | biostudies-other