Dataset Information

Ewing sarcoma outcomes in a country with limited resources: Egypt as an example.

ABSTRACT: As the scarcity of published research that comprehensively and meticulously analyzed the patient, disease, and treatment factors of prognostic significance in Ewing sarcoma (EWS) in Egypt; This study aimed at assessing survival outcomes of EWS in Upper Egypt, delineating factors of prognostic significance in comparison to other leading oncology centers in Egypt and internationally. By retrospectively reviewing medical records of 85 patients with a verified diagnosis of EWS in the period from 2001 to 2015 at Pediatric and Medical Oncology Departments at South Egypt Cancer Institute; We gathered data relevant to the patient, disease, and treatment variables of the study. Survival was estimated using the Kaplan Meier method and differences between various groups were determined by log rank test. Univariable and multivariable analyses were performed using Cox regression. With a median follow-up period of 62.7 months (95% CI 52.2-73.2, SE=5.4) for the study patients, the estimates of event-free survival (EFS) and overall survival (OS) at 3 and 5 years were 42.1% and 50.6%, and 40.8% and 48.5%, respectively. Metastatic disease at initial presentation (HR=8.91, 95% CI, 4.00-19.9; P<0.0001) stood as the most powerful predictor of OS in the multivariable analysis, followed by surgery used as a local modality (HR=0.16, 95% CI, 0.06-0.44; P=0.0004). Response to neoadjuvant chemotherapy (HR=2.61, 95% CI, 1.11-6.13; P=0.028), primary tumor size (HR=2.49, 95% CI, 1.03-6.03; P=0.044) were also shown to be significantly associated with OS. Radiotherapy as a local modality, whose effect, apparently shown to increase the hazard of events occurrence in the univariable analysis, an effect that was reversed to reveal EFS advantage (HR=0.41, 95% CI, 0.18-0.95; P=0.036) after control of other variables. With 5-year OS of 48.5%, our survival results were comparable to those previously published from Egypt; however, differences still exist between centers due to varied representative study samples. However, outcomes in Egypt in general are still inferior to internationally published studies.

SUBMITTER: Morsy AM

PROVIDER: S-EPMC8263650 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature

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Ewing sarcoma outcomes in a country with limited resources: Egypt as an example.

Morsy Ahmed Mohammed AM Abdel-Hadi Salah S Rezk Khalid Mohammed KM Amira Gamal G Ahmed Badawy Mohammed BM Hussien Marwa Tammam MT Ameen Mahmoud Gamal MG Kamel Hosam Eldein Mostafa HEM Fouad Doaa Mohamed DM Attia Alia Mohamed AM Salah Asmaa A Abd Elbadee Osama Mostafa OM Yousseif Ayatallah Ali AA Abdelgawad Marwa Ismail MI Fathy Asmaa Hussein AH Elwany Yasmine Nagy YN Ramadan Islam Karam-Allah IK Mosallam Khaled Hassan KH Abd Elwahab Ahmed Ibrahim AI Mahmoud Khaled Hashim KH Amine Maged Abdel Fattah MAF Abd Elzaher Ahmed Refaat AR Eltyb Hanan Ahmed HA Hefni Ahmed Mubarak AM

American journal of cancer research 20210615 6

As the scarcity of published research that comprehensively and meticulously analyzed the patient, disease, and treatment factors of prognostic significance in Ewing sarcoma (EWS) in Egypt; This study aimed at assessing survival outcomes of EWS in Upper Egypt, delineating factors of prognostic significance in comparison to other leading oncology centers in Egypt and internationally. By retrospectively reviewing medical records of 85 patients with a verified diagnosis of EWS in the period from 200 ...[more]

PMID: 34249456

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Project description:Adamantinoma-like Ewing sarcoma (ALES) has traditionally been considered a variant of Ewing sarcoma because it generally harbors EWSR1::FLI1 fusions despite showing diffuse positivity for keratins and p40. However, it has become increasingly recognized that different tumors can have identical translocations, including shared fusions between carcinomas and sarcomas, raising questions as to whether ALES might represent a separate entity. Using methylation profiling, we further explored the relationship between Ewing sarcoma and ALES. The archives of multiple institutions were searched for candidate cases of ALES. DNA methylation profiling was performed and results were compared to corresponding data from conventional Ewing sarcoma. Twelve cases of ALES (5 previously reported) were identified in 10 men and 2 women (aged 20-72 years; median age, 41.5 years). Cases included tumors arising in the parotid gland (3), sinonasal cavity (2), submandibular gland (2), thyroid gland (1), neck (1), gingiva (1), hypopharynx (1), and mandible (1). Histologic review consistently showed sheets and nests of basaloid cells within a fibromyxoid or hyalinized stroma. All tumors were positive for at least 1 keratin and CD99 expression, whereas all 10 cases tested were positive for p63 or p40; S100 protein expression was noted in 2 cases. Cases harbored either EWSR1::FLI1 fusions (n = 6), FUS::FLI1 fusions (n = 1), and/or EWSR1 rearrangements (n = 6). Methylation profiling was successful in 11/12 cases evaluated. Unsupervised clustering and dimensionality reduction (Uniform Manifold Approximation and Projection) of DNA methylation data revealed a distinct methylation cluster for all 11 cases, including the tumor with the FUS::FLI1 fusion, which clearly segregated them from the conventional Ewing sarcoma. Follow-up (n = 11, 1-154 months) revealed that 4 patients experienced recurrence and 6 developed metastatic disease. ALES demonstrates a distinct methylation signature from conventional Ewing sarcoma. This finding adds to the distinctive immunoprofile of ALES, suggesting that these 2 tumors should be considered distinct entities rather than histologic extremes of the same disease.

Project description:BackgroundIncreasing evidence supporting the value of screening women for cervical cancer once in their lifetime, coupled with mounting interest in scaling up successful screening demonstration projects, present challenges to public health decision makers seeking to take full advantage of the single-visit opportunity to provide additional services. We present an analytic framework for packaging multiple interventions during a single point of contact, explicitly taking into account a budget and scarce human resources, constraints acknowledged as significant obstacles for provision of health services in poor countries.Methods and findingsWe developed a binary integer programming (IP) model capable of identifying an optimal package of health services to be provided during a single visit for a particular target population. Inputs to the IP model are derived using state-transition models, which compute lifetime costs and health benefits associated with each intervention. In a simplified example of a single lifetime cervical cancer screening visit, we identified packages of interventions among six diseases that maximized disability-adjusted life years (DALYs) averted subject to budget and human resource constraints in four resource-poor regions. Data were obtained from regional reports and surveys from the World Health Organization, international databases, the published literature, and expert opinion. With only a budget constraint, interventions for depression and iron deficiency anemia were packaged with cervical cancer screening, while the more costly breast cancer and cardiovascular disease interventions were not. Including personnel constraints resulted in shifting of interventions included in the package, not only across diseases but also between low- and high-intensity intervention options within diseases.ConclusionsThe results of our example suggest several key themes: Packaging other interventions during a one-time visit has the potential to increase health gains; the shortage of personnel represents a real-world constraint that can impact the optimal package of services; and the shortage of different types of personnel may influence the contents of the package of services. Our methods provide a general framework to enhance a decision maker's ability to simultaneously consider costs, benefits, and important nonmonetary constraints. We encourage analysts working on real-world problems to shift from considering costs and benefits of interventions for a single disease to exploring what synergies might be achievable by thinking across disease burdens.

Project description:BackgroundHigh grade primary bone sarcomas are rare cancers that affect mostly children and young adults. Osteosarcoma and Ewing sarcoma are the most common histological subtypes in this age group, with current multimodality treatment strategies achieving 55-70% overall survival. As there remains an urgent need to develop new therapeutic interventions, we have reviewed published phase I/II trials that have been reported for osteosarcoma and Ewing sarcoma in the last twenty years.ResultsWe conducted a literature search for clinical trials between 1990 and 2010, either for trials enrolling bone sarcoma patients as part of a general sarcoma indication or trials specifically in osteosarcoma and Ewing sarcoma. We identified 42 clinical trials that fulfilled our search criteria for general sarcoma that enrolled these patient groups, and eight and twenty specific trials for Ewing and osteosarcoma patients, respectively. For the phase I trials which enrolled different tumour types our results were incomplete, because the sarcoma patients were not mentioned in the PubMed abstract. A total of 3,736 sarcoma patients were included in these trials over this period, 1,114 for osteosarcoma and 1,263 for Ewing sarcoma. As a proportion of the worldwide disease burden over this period, these numbers reflect a very small percentage of the potential patient recruitment, approximately 0.6% for Ewing sarcoma and 0.2% for osteosarcoma. However, these data show an increase in recent activity overall and suggest there is still much room for improvement in the current trial development structures.ConclusionLack of resources and commercial investment will inevitably limit opportunity to develop sufficiently rapid improvements in clinical outcomes. International collaboration exists in many well founded co-operative groups for phase III trials, but progress may be more effective if there were also more investment of molecular and translational research into disease focused phase I/II clinical trials. Examples of new models for early translational and early phase trial collaboration include the European based EuroBoNeT network, the Sarcoma Alliance for Research through Collaboration network (SARC) and the new European collaborative translational trial network, EuroSarc.

Dataset Information

Ewing sarcoma outcomes in a country with limited resources: Egypt as an example.

Publications

Ewing sarcoma outcomes in a country with limited resources: Egypt as an example.

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