Ewing's sarcoma / Peripheral Primitive Neuroectodermal Tumours (PNET) of bone is a type of cancer usually found in children and young adults. The peak incidence is between ages 10 and 20, it is less common in children under 5 or in adults over 30. Ewing's s can occur in any bone in the body; the most common sites are the pelvis, thigh, lower leg, upper arm, and rib. The tumour is composed of small round blue cells. Ewing's sarcoma can also arise in soft tissue (extra-skeletal); see Soft Tissue Sarcoma in this guide.Information for Patients and the Public
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MeSH term: Sarcoma, Ewing
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This list of publications is regularly updated (Source: PubMed).
miR-145 suppresses epithelial-mesenchymal transition by targeting stem cells in Ewing sarcoma cells.
Bratisl Lek Listy. 2021; 122(1):71-77 [PubMed] Related Publications
METHODS: EWS cells were utilized for functional analysis of mir-145. Proliferation, migration, invasion and soft agar colony assay were performed to observe the alterations in migration behavior of transfected cells. Caspase assay was used to investigate the underlying reasons of proliferative inhibition in cells in whichmiR-145 is overexpressed. QRT-PCR was used to determine the role of miR-145 in EMT transcription markers and mir-145 targeted genes, KLF4, SOX2 and OCT4 expression levels.
RESULTS: The miR-145 expression has been shown to be down-regulated in EWS. The miR-145 overexpression caused inhibition of proliferation and reduced migration in EWS cells through induction of apoptosis. Mir-145 suppresses EMT capacity and SOX2, KLF4 and OCT4 expression levels.
CONCLUSION: This is the first time in the literature we have shown deregulation of miR-145 inhibits EMT process by targeting stem cell properties leading to the inhibition of tumor growth and metastasis in TC71 and TC106 cells. Based on these results, we propose that miR-145, as an important regulator of SOX2, KLF4 and OCT4 carries crucial roles in EWS tumorigenesis and EMT (Tab. 1, Fig. 4, Ref. 26).
Mithramycin 2'-Oximes with Improved Selectivity, Pharmacokinetics, and Ewing Sarcoma Antitumor Efficacy.
J Med Chem. 2020; 63(22):14067-14086 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
Establishing a novel prognostic tool for Ewing sarcoma patients: Surveillance, Epidemiology, and End Results database analysis.
Medicine (Baltimore). 2020; 99(46):e23050 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
A rare entity of Primary Ewing sarcoma in kidney.
BMC Surg. 2020; 20(1):280 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
CASE PRESENTATION: Herein, we depicted a case of RES, which was administrated in our institution by chief complaints of intermittent left plank pain and palpable abdominal mass. We demonstrated the aggressive behavior of this renal malignancy and summarized its therapeutic modalities and outcomes.
CONCLUSION: The diagnosis of RES relies on integrated analysis including histomorphology, immunohistochemical staining and confirmation of molecular-genetic testing. Despite the surgery and adjuvant therapy, optimized and potent therapeutic regimes are still urgently needed to improve the poor prognosis of RES.
Inactivation of ICAM1 inhibits metastasis and improves the prognosis of Ewing's sarcoma.
J Cancer Res Clin Oncol. 2021; 147(2):393-401 [PubMed] Related Publications
METHODS: The gene expression sequence of ES metastasis samples was compared with that of primary tumor samples to obtain differentially expressed genes (DEGs). Subsequently, we annotated the gene functions and enriched pathways of DEGs. Additionally, the protein and protein interaction network were constructed to screen key genes that can lead to the metastasis in ES. Then, cell and molecular biology experiments were conducted to verify the results obtained from the bioinformatics analysis. Finally, we assessed the correlation of expression between the key genes EWSR and FLI1, and conducted a survival analysis of ICAM1.
RESULTS: Our study revealed 153 DEGs. Of these, 82 (53.59%) were upregulated and the remaining 71 (46.41%) were downregulated. The bioinformatics analysis showed that ICAM1 was the key gene leading to the invasion and metastasis of ES. Through cell biology and molecular biology experiments, inactivation of ICAM1 inhibited the metastasis of ES cells. The survival and correlation analyses showed that ICAM1 was a risk factor in patients with ES, and that ICAM1 expression was correlated with EWSR and FLI1 expression.
CONCLUSION: Our study shows that inactivation of ICAM1 inhibits metastasis and improves the prognosis of ES. Additionally, our findings provide a better understanding of the underlying mechanisms of metastatic ES, a basis for an accurate diagnosis, and therapeutic targets for ES patients.
EWS-FLI1 regulates and cooperates with core regulatory circuitry in Ewing sarcoma.
Nucleic Acids Res. 2020; 48(20):11434-11451 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
Metastatic pulmonary dissemination as differential diagnosis of COVID-19 disease.
BMJ Case Rep. 2020; 13(10) [PubMed] Article available free on PMC after 25/11/2021 Related Publications
Diagnoses, treatment, and oncologic outcomes in patients with calcaneal malignances: Case series, systematic literature review, and pooled cohort analysis.
J Surg Oncol. 2020; 122(8):1731-1746 [PubMed] Related Publications
AIMS: (1) describe the demographics of calcaneal malignancies in a large cohort; (2) describe survival after amputation versus limb-salvage surgery for high-grade tumors.
METHODS: Study group: a "pooled" cohort of patients with primary calcaneal malignancies treated at two cancer centers (1984-2015) and systematic literature review. Kaplan-Meier analyses described survival across treatment and diagnostic groups; proportional hazards modeling assessed mortality after amputation versus limb salvage.
RESULTS: A total of 131 patients (11 treated at our centers and 120 patients from 53 published studies) with a median 36-month follow-up were included. Diagnoses included Ewing sarcoma (41%), osteosarcoma (30%), and chondrosarcoma (17%); 5-year survival rates were 43%, 73% (70%, high grade only), and 84% (60%, high grade only), respectively. Treatment involved amputation in 52%, limb salvage in 27%, and no surgery in 21%. There was no difference in mortality following limb salvage surgery (vs. amputation) for high-grade tumors (HR 0.38; 95% CI 0.14-1.05), after adjusting for Ewing sarcoma diagnosis (HR 5.15; 95% CI 1.55-17.14), metastatic disease at diagnosis (HR 3.88; 95% CI 1.29-11.64), and age (per-year HR 1.04; 95% CI 1.02-1.07).
CONCLUSIONS: Limb salvage is oncologically-feasible for calcaneal malignancies.
An immune-related gene signature for determining Ewing sarcoma prognosis based on machine learning.
J Cancer Res Clin Oncol. 2021; 147(1):153-165 [PubMed] Related Publications
METHODS: We analyzed the ES gene expression profile dataset, GSE17679, from the GEO database and extracted differential expressed IRGs (DEIRGs). Then, we conducted functional correlation and protein-protein interaction (PPI) analyses of the DEIRGs and used the machine learning algorithm-iterative Lasso Cox regression analysis to build an optimal DEIRG signature. In addition, we applied ES samples from the ICGC database to test the optimal gene signature. We performed univariate and multivariate Cox regressions on clinicopathological characteristics and optimal gene signature to evaluate whether signature is an important prognostic factor. Finally, we calculated the infiltration of 24 immune cells in ES using the ssGSEA algorithm, and analyzed the correlation between the DEIRGs in the optimal gene signature and immune cells.
RESULTS: A total of 249 DEIRGs were screened and an 11-gene signature with the strongest correlation with patient prognoses was analyzed using a machine learning algorithm. The 11-gene signature also had a high prognostic value in the ES external verification set. Univariate and multivariate Cox regression analyses showed that 11-gene signature is an independent prognostic factor. We found that macrophages and cytotoxic, CD8 T, NK, mast, B, NK CD56bright, TEM, TCM, and Th2 cells were significantly related to patient prognoses; the infiltration of cytotoxic and CD8 T cells in ES was significantly different. By correlating prognostic biomarkers with immune cell infiltration, we found that FABP4 and macrophages, and NDRG1 and Th2 cells had the strongest correlation.
CONCLUSION: Overall, the IRG-related 11-gene signature can be used as a reliable ES prognostic biomarker and can provide guidance for personalized ES therapy.
Bone marrow aspirations in Ewing sarcomas: Are they still necessary? A single-center retrospective analysis and review of the literature.
J Cancer Res Ther. 2020 Jul-Sep; 16(4):713-717 [PubMed] Related Publications
Materials and Methods: This retrospective single centre study included 31 patients that were newly diagnosed with ES between 2000 and 2014. Twenty-seven patients had skeletal ES and in 4 patients the tumour was localized in the soft tissue only. Metastases at diagnosis were present in 5 out of 31 patients. BM samples were morphologically and immunohistochemically searched and screened for the presence or absence of BM metastases. Furthermore, in 15 of the 31 patients BM samples were still available and were reanalysed, using nested-polymerase chain reaction.
Results: All BM samples of our 31 ES patients, including the 5 metastatic patients, were, morphologically and immunohistochemically tested negative for tumour cell appearance. The nested-PCR results were also negative in all of our 15 retested patients, including two patients with metastatic disease.
Conclusions: Based on our results and on the contradictory results reported in the literature we recommend a re-evaluation of the necessity and the prognostic value of BMAB in the initial staging process of newly diagnosed ES patients.
Low-frequency variation near common germline susceptibility loci are associated with risk of Ewing sarcoma.
PLoS One. 2020; 15(9):e0237792 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
METHODS: We investigated the contribution of rare and low-frequency variation to EwS susceptibility in the largest EwS genome-wide association study to date (733 EwS cases and 1,346 unaffected controls of European ancestry).
RESULTS: We identified two low-frequency variants, rs112837127 and rs2296730, on chromosome 20 that were associated with EwS risk (OR = 0.186 and 2.038, respectively; P-value < 5×10-8) and located near previously reported common susceptibility loci. After adjusting for the most associated common variant at the locus, only rs112837127 remained a statistically significant independent signal (OR = 0.200, P-value = 5.84×10-8).
CONCLUSIONS: These findings suggest rare variation residing on common haplotypes are important contributors to EwS risk.
IMPACT: Motivate future targeted sequencing studies for a comprehensive evaluation of low-frequency and rare variation around common EwS susceptibility loci.
Relationship between treatment center case volume and survival for localized Ewing sarcoma: The role of radiotherapy timing.
Pediatr Blood Cancer. 2020; 67(11):e28685 [PubMed] Related Publications
Two Cases of SARS-CoV-2 Infection in Pediatric Oncohematologic Patients in Spain.
Pediatr Infect Dis J. 2020; 39(11):1040-1042 [PubMed] Related Publications
Anticancer effects of the PLK4 inhibitors CFI-400945 and centrinone in Ewing's sarcoma cells.
J Cancer Res Clin Oncol. 2020; 146(11):2871-2883 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
METHODS: CFI-400945 and centrinone were tested in three Ewing's sarcoma cell lines with different TP53 status. Effects were assessed by flow-cytometric analyses of cell death, dissipation of the mitochondrial transmembrane potential and cell cycle distribution, by cell viability assay as well as by caspase 3/7 activity measurement, by immunoblotting and by immunofluorescence microscopy.
RESULTS: CFI-400945 and centrinone elicited cell death in p53 wild-type and mutant Ewing's sarcoma cells. Both agents induced mitochondrial membrane depolarisation, caspase 3/7 activation, PARP1 cleavage and DNA fragmentation, indicating an apoptotic form of cell death. In addition, the PLK4 inhibitors induced a G2/M cell cycle arrest, particularly when cell killing was attenuated by the pan-caspase inhibitor z-VAD-fmk. Moreover, CFI-400945 treatment produced polyploidy.
CONCLUSION: Our findings show that PLK4 inhibitors were effective against Ewing's sarcoma cells in vitro and thus provide a rationale for their evaluation in vivo.
Extraskeletal, intradural, non-metastatic Ewing's sarcoma. Case report.
Ideggyogy Sz. 2020; 73(7-08):286-288 [PubMed] Related Publications
VEGFR2 as a target for CAR T cell therapy of Ewing sarcoma.
Pediatr Blood Cancer. 2020; 67(10):e28313 [PubMed] Related Publications
METHODS: Expression of VEGFR2 was studied by immunohistochemistry in human EwS biopsies and in murine xenografts and by flow cytometry in EwS cell lines. CARs with short, medium, and long hinge domains against either human or murine VEGFR2 were generated and expressed in human T cells by retroviral gene transfer. The capacity of the individual CARs to activate T cells in response to VEGFR2-expressing cells was compared in vitro.
RESULTS: Tumor-associated endothelial cells in human EwS biopsies and in xenografts expressed VEGFR2. Tumor cells in the majority of EwS biopsies were also VEGFR2-positive. Following modification with anti-mouse or anti-human VEGFR2-specific CAR genes, T cells specifically lysed VEGFR2-expressing target cells of the respective species. CAR T cells with short-length or medium-length hinge domains were functionally superior over those with the long hinge region by in vitro parameters, including antigen-specific degranulation responses, lysis of tumor spheroids, tumor necrosis factor α secretion, sequential killing, and proliferation.
CONCLUSIONS: VEGFR2 is consistently expressed on endothelial cells of the tumor stroma in EwS and thus is a candidate target for CAR T cells in this cancer. Among various VEGFR2-specific CARs, a construct with a short hinge domain was chosen to be further developed toward clinical translation.
The clinical prognostic factors and treatment outcomes of adult patients with Ewing sarcoma.
Int J Clin Oncol. 2020; 25(11):2006-2014 [PubMed] Related Publications
METHODS: Between 2000 and 2018, 180 patients at the age of > 18 years old diagnosed with Ewing sarcoma were treated in referral center according to multimodal protocols. In 50 patients (28%) treatment was initiated outside our hospital, and 23 of them had started recommended therapy after 3 months since the date of biopsy/unscheduled operation. We analyzed clinical prognostic factors and overall survival (OS).
RESULTS: The median age was 28 years (18-67 years), primary tumor was localized axially in 114 patients (63%), metastases at presentation were detected in 51 pts (28%). 5-year OS rate was 65% for patients with localized disease, in metastatic disease it was 15%; the presence and the number of metastases was a prognostic factor. 5-year PFS was significantly better in patients treated at referral center (or when the patients were admitted to referral center within 3 months from the date of biopsy, which was performed outside referral center), comparing to patients treated initially outside referral center; 5-year PFS rates in total population were 28 and 13%, respectively. In terms of OS, unfavorable prognostic factor showing a statistical trend (p = 0.098) was lower dose density of neoadjuvant chemotherapy due to toxicity.
CONCLUSIONS: Approximately two-third of adult patients with localized Ewing sarcoma survive 5 years. In order to improve survival of this patients the multidisciplinary treatment in referral center is mandatory.
Multiparametric MRI with diffusion-weighted imaging in predicting response to chemotherapy in cases of osteosarcoma and Ewing's sarcoma.
Br J Radiol. 2020; 93(1115):20200257 [PubMed] Related Publications
METHODS: The study included 104 patients pathologically proved osteosarcoma (53) and Ewing`s sarcoma (51) underwent MRI examinations; before and after chemotherapy. All patients were assessed using the RECIST 1.1 criteria, m-RECIST, quantitative ADC, and tumor volume evaluation. 21 patients underwent DCE-MRI curve type with quantitative parameters. Correlation between the different evaluations was carried out. Results were correlated with the post-operative pathology in 42 patients who underwent surgery and for statistical evaluation, Those patients were classified into responders (≥90% necrosis) and non-responders (<90% necrosis).
RESULTS: The initial mean ADC of 104 patients of osteosarcoma and Ewing's sarcoma (0.90 ± 0.29) and (0.71 ± 0.16) respectively, differed significantly from that after treatment (1.62 ± 0.46) and (1.6 ± 0.39) respectively with (
CONCLUSION: Quantitative diffusion-weighted imaging with ADC mapping and ADC % after chemotherapy allows a detailed analysis of the treatment response in osteosarcoma and Ewing's sarcoma. The therapeutic response can be underestimated using RECIST 1.1, so the modified RECIST should be also considered.
ADVANCES IN KNOWLEDGE: Quantitative ADC especially ADC% provided an accurate non-invasive tool in the assessment of post-therapeutic cases of osteosarcoma and Ewing's sarcoma.
Outcomes with nondose-dense chemotherapy for Ewing sarcoma: A practical approach for the developing world.
Pediatr Blood Cancer. 2020; 67(11):e28604 [PubMed] Related Publications
PROCEDURE: A retrospective analysis was conducted of patients (<15 years) with Ewing sarcoma treated with curative intent during January 2013-June 2017 with an institutional ethics committee-approved nondose-dense protocol (EFT-2001). Local therapy was planned after 9-12 weeks of chemotherapy with metastatic sites addressed with radiotherapy. The study assessed outcomes and prognostic factors.
RESULTS: We analysed 200 patients with M:F ratio of 1.27:1 and metastases in 41 patients (20.5%). At a median follow up of 41.5 months (range 4.5-81.8 months), respective 3-year EFS and overall survival (OS) of the whole cohort is 65.3% (95% confidence interval [CI]: 58.1-71.7%) and 79.3% (95% CI: 72.8-84.5%); for localized and metastatic cohort, 70.9% (95% CI: 62.9-77.5%) and 82.8% (95% CI: 75.7-89.0%); and for metastatic cohort, 42.8% (95% CI: 28.0-58.6%) and 65.3% (95% CI: 47.7-78.3%). Presence of residual disease (morphologic/metabolic) on positron emission tomography-computed tomography scan done 3 months post definitive radiotherapy (hazard ratio [HR] 7.92 [95% CI: 3.46-18.14]) and delay in any form of local control >4 months (HR 3.42 [95% CI: 1.32-8.89]) affected outcomes. Nonrelapse mortality during treatment was 6.5%, mainly due to cardiomyopathy (3.0%) and bacterial sepsis (1.5%). Cardiotoxicity was seen in 11.5% of patients.
CONCLUSIONS: Nondose-dense chemotherapy provides good outcomes with manageable toxicities in a multidisciplinary treatment approach, while reducing cumulative drug exposures in the developing world where dose-intense or dose-dense chemotherapy could potentially increase toxicity, and hence seems a feasible approach in resource-limited settings. Presence of any residual disease post definitive radiotherapy or delay in local control portends poor outcome.
Pediatr Blood Cancer. 2020; 67(11):e28605 [PubMed] Related Publications
OBJECTIVE: To determine the potential role of
METHODS: Thirty-six patients who were treated with NAC and surgery at the Children's Cancer Hospital, Egypt, were prospectively included in this study. All patients underwent two studies; a PET/CT study before NAC and another one after NAC completion. Metabolic PET parameters were measured in each study. The ability of each of these parameters, their pretreatment and pre-local control values, as well as the percentage reduction between their pretreatment and pre-local control values, were evaluated to differentiate between good and poor responders using the histological response as a standard reference.
RESULTS: Neither the pretreatment value nor the percentage reduction of any of the measured PET parameters predicted poor histological response. After NACcompletion, metabolic tumor volume (MTV) at the threshold of an SUV of 2.5 isocontour (MTV(2.5)
CONCLUSION: FDG PET parameters can predict poor histological response to NAC in ESFT patients. MTV and TLG at the thresholds of an SUV of 2.5 isocontour and hepatic reference SUVmean are the two most promising thresholds in predicting the response of patients. The cutoff value of SUVmax
Exchange Cranioplasty Using Bioabsorbable Hydroxyapatite and Collagen Complex After Removal of an Extensive Frontal Bone Tumor in an Infant.
World Neurosurg. 2020; 142:375-378 [PubMed] Related Publications
CASE DESCRIPTION: A 4-month-old girl was presented with a right frontal bone Ewing sarcoma. The tumor was removed, and the defect was reconstructed with an autologous contralateral parietal bone graft. The parietal bone defect was repaired with a bioabsorbable hydroxyapatite and collagen complex. Good reconstruction of the forehead and ossification of the donor site was achieved within 3 years after surgery.
CONCLUSIONS: After removal of an extensive frontal bone tumor in an infant, exchange cranioplasty with an autograft using a bioabsorbable hydroxyapatite and collagen complex at the donor site yielded good results.
Avoiding Limb-Length Discrepancy with Reconstruction of a Massive Tibial Defect Using a Bone Allograft and a Minimally Invasive Lengthening System in a Pediatric Patient: A Case Report.
JBJS Case Connect. 2020 Apr-Jun; 10(2):e0456 [PubMed] Related Publications
CONCLUSIONS: Limb-salvage reconstruction in children can be complicated by the sacrifice of epiphyseal plates and limb-length discrepancies and thus requires techniques tailored to each case.
Primary vaginal Ewing sarcoma with uterine fibroid: A case report.
Medicine (Baltimore). 2020; 99(27):e20859 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
PATIENT CONCERNS: The patient was admitted to our hospital because of vaginal pain. Her uterus showed as parallel position and enlarged as about 3 months of pregnancy size.
DIAGNOSIS: Magnetic resonance imaging (MRI) and ultrasonography (US) demonstrated 2 heterogeneous masses in the vagina and uterus, respectively. Ultrasound-guided puncture biopsy revealed a malignant tumor in the right lateral vaginal wall.
INTERVENTIONS: The patient was treated by hysterectomy, bilateral salpingo-oophorectomy, and tumors excision, with the subsequent treatment of chemotherapy.
OUTCOMES: The patient recovered well without local recurrence for >1 year.
LESSONS: Primary vaginal Ewing sarcoma is extremely rare. The treatments of uterine fibroid include uterine artery embolization and surgical options, While wide local excision followed by adjuvant chemotherapy and/or radiotherapy should be recommended for the vaginal ES.
Challenges in the management of localized Ewing sarcoma in a developing country.
Pediatr Hematol Oncol. 2020; 37(7):610-619 [PubMed] Related Publications
Phase II results from a phase I/II study to assess the safety and efficacy of weekly nab-paclitaxel in paediatric patients with recurrent or refractory solid tumours: A collaboration with the European Innovative Therapies for Children with Cancer Network.
Eur J Cancer. 2020; 135:89-97 [PubMed] Related Publications
PATIENTS AND METHODS: Paediatric patients with recurrent or refractory Ewing sarcoma, neuroblastoma or rhabdomyosarcoma received 240 mg/m
RESULTS: Forty-two patients were enrolled, 14 each with Ewing sarcoma, neuroblastoma and rhabdomyosarcoma. The ORRs were 0%, 0% and 7.1% (1 confirmed PR), respectively. The DCRs were 30.8% (4 SD), 7.1% (1 SD) and 7.1% (1 confirmed PR and 0 SD) in the Ewing sarcoma, neuroblastoma and rhabdomyosarcoma groups, respectively. The median progression-free survival was 13.0, 7.4 and 5.1 weeks, respectively, and the 1-year overall survival rates were 48%, 25% and 15%, respectively. The most common grade III/4IVadverse events were haematologic (neutropenia [50%] and anaemia [48%]), and grade III/IV peripheral neuropathy occurred in 2 patients (14%) in the rhabdomyosarcoma group. Pharmacokinetics analyses revealed that paclitaxel tissue distribution was both rapid and extensive.
CONCLUSIONS: In this phase II study, limited activity was observed; however, the safety of nab-paclitaxel in paediatric patients was confirmed.
TRIAL REGISTRATION: NCT01962103 and EudraCT 2013-000144-26.
Evaluation of factors affecting survival rate in primary bone sarcomas with extremity and pelvis involvement.
Acta Orthop Traumatol Turc. 2020; 54(3):234-244 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
METHODS: A total of 114 patients (64 male and 50 female) with osteosarcoma, chondrosarcoma, or Ewing's sarcoma treated between 2005 and 2013 were included in this study. All the patients received standard treatment and were followed up regularly. In all, 44 cases of (conventional and telangiectatic) osteosarcoma, 30 cases of Ewing's sarcoma, and 40 cases of high-grade chondrosarcoma were identified using the Bone and Soft Tissue Tumor Registry. Gender, age, tumor size and localization, pathological fractures, histopathological type, grade, surgical treatment, adjuvant treatments, relapse of the disease, and postoperative complication data were obtained from follow-up forms. The learning curve of institutional expertise was also evaluated. The patient survival rate was calculated using the Kaplan-Meier method, and log-rank statistical methods were used to compare survival rates.
RESULTS: The mean length of survival of the patients was 72 months. There was a 56% 5-year survival rate, and the event-free survival rate was 53%. The survival of patients with Ewing's sarcoma whose prodromal period was less than 12 weeks was significantly higher than that of the other groups (p=0.031). The survival of patients with tumor size greater than 150 cc, with local recurrence and distant metastases was low for all groups. Survival rates were significantly lower in osteosarcoma and Ewing's sarcoma patients with stage III tumor or metastasis at diagnosis. The survival of patients with osteosarcoma diagnosed between 2010 and 2013 was significantly higher than that of the earlier group (p=0.02).
CONCLUSION: Decreasing the prodromal period (early diagnosis) can improve survival by preventing the local and systemic spread of the tumor. Increase in the surgical experience is likely to have a positive effect on survival rates, especially for patients with osteosarcoma. The relapse of the disease is a poor prognostic factor for survival despite aggressive surgery and adjuvant therapies.
LEVEL OF EVIDENCE: Level IV, Therapeutic study.
An inducible ectopic expression system of EWSR1-FLI1 as a tool for understanding Ewing sarcoma oncogenesis.
PLoS One. 2020; 15(6):e0234243 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
Up-regulation of GLI1 in vincristine-resistant rhabdomyosarcoma and Ewing sarcoma.
BMC Cancer. 2020; 20(1):511 [PubMed] Article available free on PMC after 25/11/2021 Related Publications
METHODS: We characterized baseline expression and activity of HH pathway components in 5 RMS (RD, Rh18, Ruch-2, Rh30, and Rh41) and 5 EWS (CHLA9, CHLA10, TC32, CHLA258, and TC71) cell lines. We then established VCR-resistant RMS and EWS cell lines by exposing cells to serially increasing concentrations of VCR and determining the IC
RESULTS: We found evidence for HH pathway activity and GLI1 expression in RMS and EWS cell lines at baseline, and evidence that GLI1 contributes to survival and proliferation of these sarcoma cells. We were able to establish 4 VCR-resistant cell lines (Ruch-2VR, Rh30VR, Rh41VR, and TC71VR). GLI1 was significantly up-regulated in the Rh30VR, Rh41VR, and TC71VR cells. The only other gene in the drug resistance panel that was significantly up-regulated in each of these VCR-resistant cell lines compared with their corresponding parental cells was the GLI1 direct target and multidrug resistance gene, ATP-binding cassette sub-family B member 1 (MDR1). We established major vault protein (MVP), which was up-regulated in both vincristine-resistant alveolar RMS cell lines (Rh30VR and Rh41VR), as another direct target of GLI1 during development of drug resistance. Treatment of the VCR-resistant cell lines with the small molecule inhibitor GANT61 or GLI1 siRNA together with VCR significantly decreased cell viability at doses that did not reduce viability individually.
CONCLUSIONS: These experiments demonstrate that GLI1 up-regulation contributes to VCR resistance in RMS and EWS cell lines and suggest that targeting GLI1 may benefit patients with RMS or EWS by reducing multidrug resistance.
Image-guided core needle biopsy for Ewing sarcoma of bone: a 10-year single-institution review.
Eur Radiol. 2020; 30(10):5308-5314 [PubMed] Related Publications
METHODS: All patients with a confirmed diagnosis of Ewing sarcoma who underwent IGCNB between January 2007 and December 2016 were included in this retrospective study. Analysis included mean age, skeletal distribution, imaging modality used for biopsy guidance, type of anaesthesia, needle type, number of passes, type of tissue sampled, and complications.
RESULTS: The study included 139 patients (94 males and 45 females; mean age 18.7 years) who underwent 141 image-guided core needle biopsies as the primary diagnostic test. Of these, 101 were CT-guided, 38 ultrasound-guided, and 2 utilised both CT and ultrasound guidance. A total of 97.9% were diagnostic at first procedure. Of the 3 non-diagnostic cases, 2 underwent a further IGCNB and were positive, while 1 patient required an open surgical procedure. Only 1 patient (0.7%) suffered an immediate complication, and there were no recorded delayed complications.
CONCLUSION: IGCNB is a safe procedure providing a positive diagnosis of Ewing sarcoma of bone in a very high percentage of cases. It should be the first-line method for establishing a diagnosis in suspected Ewing sarcoma of bone.
KEY POINTS: • Image-guided core needle biopsy is a safe procedure providing a positive diagnosis of Ewing sarcoma of bone in a very high percentage of cases. • Image-guided core needle biopsy should be the first-line method for establishing a definitive diagnosis in Ewing sarcoma and should be performed at a specialist sarcoma referral centre. • When technically feasible, extra-osseous soft tissue alone can be sampled with confidence as there is no difference in diagnostic performance whether bone or an extra-osseous soft tissue component of the tumour is sampled.