Ewing’s Sarcoma

Riggi, Nicolò; Suvà, Mario L.; Stamenkovic, Ivan

doi:10.1056/nejmra2028910

Cited by 212 publications

(272 citation statements)

References 90 publications

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“…Human Ewing sarcoma can occur in any part of the body in bone or soft tissue, but not endodermally or ectodermally derived tissue. It most commonly involves the pelvis and proximal long bones (Riggi et al, 2021). In our model, based on mosaic integration of human EWSR1-FLI1 into the zebrafish genome, formation of tumors was observed in association with mesenchymally derived skeleton at the regions proximal to the base of pectoral, anal and caudal fins, and at supraneurals.…”

Section: Discussionmentioning

confidence: 93%

“…Although Ewing sarcoma was first described over years ago, there are few if any molecularly targeted therapies for patients with metastatic or relapsed disease. Fewer than 30% of patients presenting with metastases survive for 5 years (Riggi et al, 2021). While great progress has been made using cells, xenografts and PDX models, the development of complementary Ewing sarcoma animal models remains crucial for the understanding of disease biology in the complex developmental microenvironment.…”

Section: Discussionmentioning

confidence: 99%

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Dysregulated heparan sulfate proteoglycan metabolism promotes Ewing sarcoma tumor growth

Vasileva

Warren

et al. 2021

Preprint

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The Ewing sarcoma family of tumors is a group of malignant small round blue cell tumors (SRBCTs) that affects children, adolescents and young adults. The tumors are characterized by reciprocal chromosomal translocations that generate chimeric fusion oncogenes, the most common of which is EWSR1-FLI1. Survival is extremely poor for patients with metastatic or relapsed disease, and no molecularly-targeted therapy for this disease currently exists. The absence of a reliable genetic animal model of Ewing sarcoma has impaired investigation of tumor cell/microenvironmental interactions in vivo. We have developed a new genetic model of Ewing sarcoma based on Cre-inducible expression of human EWSR1-FLI1 in wild type zebrafish, which causes rapid onset of SRBCTs at high penetrance. The tumors express canonical EWSR1-FLI1 target genes and stain for known Ewing sarcoma markers including CD99. Growth of tumors is associated with activation of the MAPK/ERK pathway, which we link to dysregulated extracellular matrix metabolism in general and heparan sulfate catabolism in particular. Targeting heparan sulfate proteoglycans with the specific heparan sulfate antagonist Surfen reduces ERK1/2 signaling and decreases tumorigenicity of Ewing sarcoma cells in vitro and in vivo. These results highlight the important role of the extracellular matrix in Ewing sarcoma tumor growth and the potential of agents targeting proteoglycan metabolism as novel therapies for this disease.

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Section: Discussionmentioning

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Dysregulated heparan sulfate proteoglycan metabolism promotes Ewing sarcoma tumor growth

Vasileva

Warren

et al. 2021

Preprint

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“…ES is an aggressive disease with a 70% overall 5-year relative survival rate [3]. However, the difference in [18].…”

Section: Discussionmentioning

confidence: 99%

“…ES is a member of the so-called "small round blue cell" tumors, most commonly arising in bone and most frequently found in children and adolescents. Extraosseous Ewing sarcoma (EES) is less frequent and can occur at a wide variety of anatomic [3]. Several recent reports have described an adamantinoma-like Ewing sarcoma (ALES) arising in the thyroid gland, based on the identification of EWSR1 rearrangement in neoplasms showing the classic ES morphology with epithelial differentiation (keratin and p63 or p40 expression) [4][5][6][7][8], and carcinoma of the thyroid with Ewing family tumor elements (CEFTEs) when associated with papillary thyroid carcinoma [9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

Primary Extra-Osseous Ewing Sarcoma of the Thyroid: A Case Report and Review of the Literature

Seipel

Mechahougui

Mach

et al. 2021

Head and Neck Pathol

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Extra-osseous Ewing sarcoma (ES) is a rare and aggressive malignant tumor found in a variety of organs. Primary ES of the thyroid is exceedingly rare and few cases have been documented to date. We describe the case of a 54-year old woman with a history of breast carcinoma in whom a unique hypermetabolic left thyroid nodule was identified during a follow-up PET-CT scan. An ultrasound examination showed a hypoechogenic nodule of 3.7 cm. A cytological diagnosis of poorly differentiated thyroid carcinoma was made, and a total thyroidectomy was performed. The surgical specimen revealed a poorly differentiated neoplasm composed of medium-sized cells with scant cytoplasm, expressing pancytokeratin, CD99 and NKX2.2 but lacking p63 and p40 expression. Molecular analysis revealed a EWSR1-FLI1 fusion transcript supporting the diagnosis of a primary extra-osseous ES of the thyroid. The patient received adjuvant chemotherapy and has no evidence of recurrent disease.

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“…[22]. Первоначально опухоль была названа эндотелиомой кости, так как предполагалось, что она возникла из кровеносных сосудов костной ткани [23].…”

Section: классификация опухолей костейunclassified

Expression of the immune checkpoint B7-H3 in tumor and its soluble form in serum of patients with bone neoplasms

Kushlinskii¹,

Ковалева²,

Alferov³

et al. 2021

Alʹm. klin. med.

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B7-H3, also called CD276, is a type I transmembrane glycoprotein that is encoded on human chromosome 15. It was discovered back in 2001. The original study described it as a positive co-stimulant, as it can stimulate T-cell response and IFN-γ production. However, recent researches have shown that B7-H3 is involved in T-cell inhibition. A B7-H3 receptor has not been yet identified, and this may explain the complex immunomodulatory activity of B7-H3, as it can have more than one binding partner with different functions. Expression of the B7-H3 protein has been found on activated immune cells such as T-cells, NK cells and antigen presenting cells. Interestingly, it is overexpressed in a wide range of tumor cells and is associated with disease progression and outcome. The soluble form of this protein is also of particular interest. Increased sB7-H3 levels in the plasma of bone tumor patients might be their important diagnostic criterion.

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Ewing’s Sarcoma

Cited by 212 publications

References 90 publications

Dysregulated heparan sulfate proteoglycan metabolism promotes Ewing sarcoma tumor growth

Dysregulated heparan sulfate proteoglycan metabolism promotes Ewing sarcoma tumor growth

Primary Extra-Osseous Ewing Sarcoma of the Thyroid: A Case Report and Review of the Literature

Expression of the immune checkpoint B7-H3 in tumor and its soluble form in serum of patients with bone neoplasms

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