Extraskeletal Ewing sarcoma: Diagnosis, management and prognosis (Review)

Abboud, Abdallah; Masrouha, Karim; Saliba, Maelle; Haidar, Rachid; Saab, Raya; Khoury, Nabil; Tawil, Ayman; Saghieh, Saïd

doi:10.3892/ol.2021.12615

Cited by 70 publications

(75 citation statements)

References 42 publications

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“…Metastasis predominantly occurs in the lungs, bones, and the bone marrow (5). ES has dismal prognosis, exhibiting a five-year survival rate of 70-80%, which worsens to 30% in patients with metastatic tumors (6)(7)(8). Therefore, understanding occurrence and the underlying mechanism of ES development will aid in identification of novel biomarkers for development of early diagnostic and treatment therapies, thereby improving and reducing survival and recurrence rates, respectively.…”

Section: Introductionmentioning

confidence: 99%

LncRNA FOXP4-AS1 Promotes Progression of Ewing Sarcoma and Is Associated With Immune Infiltrates

Xiong

et al. 2021

Front. Oncol.

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Ewing sarcoma (ES) is a highly malignant primary bone tumor with poor prognosis. Studies have shown that abnormal expression of lncRNA influences the prognosis of tumor patients. Herein, we established that FOXP4-AS1 was up-regulated in ES and this correlated with poor prognosis. Further analysis illustrated that FOXP4-AS1 down-regulation repression growth, migration, along with invasion of ES. On the contrary, up-regulation of FOXP4-AS1 promoted the growth, migration, as well as invasion of ES. To explore the mechanism of FOXP4-AS1, Spearman correlation analysis was carried out to determine genes that were remarkably linked to FOXP4-AS1 expression. The potential functions and pathways involving FOXP4-AS1 were identified by GO analysis, Hallmark gene set enrichment analysis, GSEA, and GSVA. The subcellular fractionation results illustrated that FOXP4-AS1 was primarily located in the cytoplasm of ES cells. Then a ceRNA network of FOXP4-AS1 was constructed. Analysis of the ceRNA network and GSEA yielded two candidate mRNAs for FOXP4-AS1. Results of the combined survival analysis led us to speculate that FOXP4-AS1 may affect the expression of TMPO by sponging miR-298, thereby regulating the malignant phenotype of ES. Finally, we found that FOXP4-AS1 may modulates the tumor immune microenvironment in an extracellular vesicle-mediated manner. In summary, FOXP4-AS1 correlates with poor prognosis of ES. It promotes the growth, migration, as well as invasion of ES cells and may modulate the tumor immune microenvironment.

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Section: Introductionmentioning

confidence: 99%

LncRNA FOXP4-AS1 Promotes Progression of Ewing Sarcoma and Is Associated With Immune Infiltrates

Xiong

et al. 2021

Front. Oncol.

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“…Primary intracranial EWS/pPNET and cPNE are both primitive ectoderm tumors. However, the cell-oforigin is different, cPNET originates from granular cells in the outer cerebellum, subventricular stromal cells of the fourth ventricle, or pineal precursor cells [17]. Primary intracranial EWS/pPNET is mainly derived from the dura mater, and the cells of its origin are not yet clear.…”

Section: Discussionmentioning

confidence: 99%

“…Chemotherapy is one of the most important treatments for primary intracranial EWS/pPNET. A previous study showed that adjuvant chemotherapy improved the 5-year survival rate from 5 to 10% to >65% [17,20]. Current systemic chemotherapy drugs include vincristine-doxorubicin-cyclophosphamide and dactinomycin alternating with ifosfamideetoposide [10].…”

Section: Discussionmentioning

confidence: 99%

Primary Intracranial Ewing Sarcoma/peripheral primitive neuroectodermal tumor: A Case Report

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Wang

Huang

et al. 2022

Preprint

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Background: Primary intracranial Ewing sarcoma/peripheral primitive neuroectodermal tumor (EW S/pPNET) is exceedingly rare; and easy to misdiagnose.Case presentation: We report a case of a 23-year-old male who presented with headache and vomiting. The preoperative imaging examination of the brain revealed an irregular mass in the left parietal lobe, which was misdiagnosed as a meningioma, but the surgical specimen was diagnosed as primary intracranial EWS/pPNET. The patient underwent a total tumor resection, followed by adjuvant chemotherapy and radiotherapy. No recurrence or distant metastasis was found 18 months post-surgery. In addition, the clinical manifestations and treatment prognosis of primary intracranial EWS/pPNET were explored through a literature review.Conclusions: When the imaging features of young patients’ lesions are solid, aggressive, and unevenly enhanced masses, physicians should be aware of the possibility of primary intracranial EWS/pPNET, and if possible, gross total resection (GTR) and intensive chemotherapy and radiotherapy are recommended.

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“…1 The incidence of such "extraosseous" ES is reportedly around 0.4 per million population, roughly 10 times less than that of ES in bones. [3][4][5] The pathological and molecular appearance of extraosseous ES seems indistinguishable from that of its counterpart in bone, 4 which has been amply described. 6 Less is known, however, about its clinical behavior and the optimal approach to its treatment, as fewer studies have focused specifically on extraosseous ES.…”

Section: Introductionmentioning

confidence: 99%

Extraosseous Ewing sarcoma in children and adolescents: A retrospective series from a referral pediatric oncology center

Livellara

Bergamaschi

Puma

et al. 2021

Pediatric Blood & Cancer

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Background: Extraosseous Ewing sarcoma is a rare entity and less is known about its clinical behavior and optimal treatment than for its counterpart in bone. This study is a retrospective analysis on a cohort of patients <21 years treated according to a "soft tissue sarcoma approach." Methods:The "extraosseous" origin of the tumor was established on radiological findings, based on the lack of any bone involvement. Patients were treated using a multimodality approach including surgery, radiotherapy, and chemotherapy. All patients received chemotherapy with alkylating agents and anthracyclines for 25 weeks (nine courses). Radiotherapy (45-54.8 Gy) was required for all cases except those who had an initial R0 resection of tumors smaller than 5 cm.Results: Fifty-seven patients (age 2-20 years, median 14) were treated from 1990 to 2020. Ten-year event-free survival (EFS) and overall survival (OS) were 77.5% and 85.5% in patients with localized disease, and 11.1% and 29.6% in those with metastatic disease (p < .001) (follow-up 5-349 months, median 107 months). In patients with localized disease, the most recent IVADo-IVE regimen achieved excellent survivals, that is, 10-year EFS 95.5%. Conclusions:Our study showed that satisfactory results were achieved in patients with localized extraosseous Ewing sarcoma treated with a tailored approach derived from soft tissue sarcoma protocols, which was less intensive and shorter as compared to the standards utilized for the management of bone Ewing sarcoma. Our study suggests that the extraskeletal site might be considered as a variable to stratify patients and modulate treatment intensity accordingly in Ewing sarcoma protocol.

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Extraskeletal Ewing sarcoma: Diagnosis, management and prognosis (Review)

Cited by 70 publications

References 42 publications

LncRNA FOXP4-AS1 Promotes Progression of Ewing Sarcoma and Is Associated With Immune Infiltrates

LncRNA FOXP4-AS1 Promotes Progression of Ewing Sarcoma and Is Associated With Immune Infiltrates

Primary Intracranial Ewing Sarcoma/peripheral primitive neuroectodermal tumor: A Case Report

Extraosseous Ewing sarcoma in children and adolescents: A retrospective series from a referral pediatric oncology center

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