Pediatric Tumors: Extraskeletal Ewing Sarcoma

2017 ◽  
Author(s):  
John Groundland ◽  
Sara Shaw
Keyword(s):  
Ewing Sarcoma ◽  
Survival Data ◽  
Molecular Basis ◽  
Gene Rearrangement ◽  
Cytotoxic Chemotherapy ◽  
Current Therapy ◽  
Pediatric Tumors ◽  
Rare Clinical Entity ◽  
Therapeutic Radiation ◽  
Extraskeletal Ewing Sarcoma

Extraskeletal Ewing sarcoma is a rare clinical entity under the umbrella of the Ewing sarcoma family of tumors. The pathogenesis of the tumor has yet to be fully described, but a gene rearrangement and the resultant fusion protein characterize the molecular basis of the disease. Current therapy centers on cytotoxic chemotherapy and local control, either through surgical resection or therapeutic radiation. Survival data specific to extraskeletal Ewing sarcoma are limited due to the rare nature and varied presentation of the disease but parallel those of skeletally based Ewing sarcoma. This review contains 14 figures, 5 tables and 50 references.  Key words: CD 99, Ewing sarcoma, EWS-FLI1, extraskeletal Ewing sarcoma, pediatric soft tissue sarcoma, t(11;22) 

Pediatric Tumors: Extraskeletal Ewing Sarcoma

2017 ◽  
Author(s):  
John Groundland ◽  
Sara Shaw
Keyword(s):  
Ewing Sarcoma ◽  
Survival Data ◽  
Molecular Basis ◽  
Gene Rearrangement ◽  
Cytotoxic Chemotherapy ◽  
Current Therapy ◽  
Pediatric Tumors ◽  
Rare Clinical Entity ◽  
Therapeutic Radiation ◽  
Extraskeletal Ewing Sarcoma

Extraskeletal Ewing sarcoma is a rare clinical entity under the umbrella of the Ewing sarcoma family of tumors. The pathogenesis of the tumor has yet to be fully described, but a gene rearrangement and the resultant fusion protein characterize the molecular basis of the disease. Current therapy centers on cytotoxic chemotherapy and local control, either through surgical resection or therapeutic radiation. Survival data specific to extraskeletal Ewing sarcoma are limited due to the rare nature and varied presentation of the disease but parallel those of skeletally based Ewing sarcoma. This review contains 14 figures, 5 tables and 50 references.  Key words: CD 99, Ewing sarcoma, EWS-FLI1, extraskeletal Ewing sarcoma, pediatric soft tissue sarcoma, t(11;22) 

Pediatric Tumors: Rhabdomyosarcoma

2017 ◽  
Author(s):  
John Groundland ◽  
Sara Shaw ◽  
R L Randall
Keyword(s):  
Skeletal Muscle ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Cytotoxic Chemotherapy ◽  
Embryonal Rhabdomyosarcoma ◽  
Pediatric Tumors ◽  
Skeletal Muscle Differentiation ◽  
Systemic Control ◽  
Therapeutic Radiation ◽  
Pleomorphic Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is a group of soft tissue sarcomas that share a common feature of primitive skeletal muscle differentiation. Three main subtypes have been characterized: embryonal, alveolar, and pleomorphic. Presentation and prognosis of RMS are highly variable, depending on anatomic location, subtype, and risk stratification. Currently, treatment centers on systemic control with cytotoxic chemotherapy and local control with surgery and/or therapeutic radiation. This review contains 3 figures, 10 tables and 50 references  Key words: alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, pediatric soft tissue sarcoma, pleomorphic rhabdomyosarcoma, rhabdomyosarcona, t(2;13), t(1;13)

Pediatric Tumors: Rhabdomyosarcoma

2017 ◽  
Author(s):  
John Groundland ◽  
Sara Shaw ◽  
R L Randall
Keyword(s):  
Skeletal Muscle ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Cytotoxic Chemotherapy ◽  
Embryonal Rhabdomyosarcoma ◽  
Pediatric Tumors ◽  
Skeletal Muscle Differentiation ◽  
Systemic Control ◽  
Therapeutic Radiation ◽  
Pleomorphic Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is a group of soft tissue sarcomas that share a common feature of primitive skeletal muscle differentiation. Three main subtypes have been characterized: embryonal, alveolar, and pleomorphic. Presentation and prognosis of RMS are highly variable, depending on anatomic location, subtype, and risk stratification. Currently, treatment centers on systemic control with cytotoxic chemotherapy and local control with surgery and/or therapeutic radiation. This review contains 3 figures, 10 tables and 50 references  Key words: alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, pediatric soft tissue sarcoma, pleomorphic rhabdomyosarcoma, rhabdomyosarcona, t(2;13), t(1;13)

Extraskeletal Ewing Sarcoma of Cervical Epidural Region: Cases Report

2003 ◽  
Vol 48 (1) ◽  
pp. 85 ◽  
Author(s):  
Kijun Kim ◽  
Hyun Seouk Jung ◽  
Jae Hee Lee ◽  
Kyung Myung Sohn ◽  
Sung Yong Lee
Keyword(s):  
Ewing Sarcoma ◽  
Extraskeletal Ewing Sarcoma

Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature

2011 ◽  
Vol 285 (3) ◽  
pp. 785-789 ◽  
Author(s):  
Katharina Kelling ◽  
Frank Noack ◽  
Christopher Altgassen ◽  
Peter Kujath ◽  
Michael K. Bohlmann ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Review Of The Literature ◽  
Extraskeletal Ewing Sarcoma

scholarly journals Extraskeletal Ewing sarcoma attached to the ulnar nerve: A case report

2021 ◽  
Vol 80 ◽  
pp. 105676
Author(s):  
Tomohiko Sakuda ◽  
Taisuke Furuta ◽  
Muhammad Phetrus Johan ◽  
Koji Arihiro ◽  
Nobuo Adachi
Keyword(s):  
Case Report ◽  
Ulnar Nerve ◽  
Ewing Sarcoma ◽  
Extraskeletal Ewing Sarcoma

scholarly journals Translational evidence for RRM2 as a prognostic biomarker and therapeutic target in Ewing sarcoma

2021 ◽  
Author(s):  
Shunya Ohmura ◽  
Aruna Marchetto ◽  
Martin F. Orth ◽  
Jing Li ◽  
Susanne Jabar ◽  
...  
Keyword(s):  
Overall Survival ◽  
Ewing Sarcoma ◽  
Survival Data ◽  
Therapeutic Target ◽  
Prognostic Biomarker ◽  
Specific Inhibitor ◽  
Cell Cycle Checkpoint ◽  
Regulatory Subunit

Purpose: Ewing sarcoma (EwS) is a highly aggressive bone- or soft tissue-associated malignancy mostly affecting children, adolescents, and young adults. Although multimodal therapies have strongly improved patients′ overall survival over the past decades, the development of prognostic biomarkers for risk-based patient stratification and more effective therapies with less adverse effects is stagnating. Thus, new personalized medicine approaches are urgently required. Experimental design: Gene expression data of EwS and normal tissues were crossed with survival data to identify highly overexpressed, prognostically relevant, and actionable potential targets. RNA-interference and dose-response assays as well as tissue-microarray analyses were carried out to explore the functional role and druggability of a prominent candidate gene in vitro and in vivo, and to validate its suitability as a prognostic biomarker. Results: Employing a multilayered screening approach, we discover ribonucleotide reductase regulatory subunit M2 (RRM2) as a promising therapeutic target and prognostic biomarker in EwS. Through analysis of two independent EwS patient cohorts, we show that RRM2 mRNA and protein overexpression is associated with an aggressive clinical phenotype and poor patients′ overall survival. In agreement, RRM2 silencing as well as pharmacological inhibition by the specific inhibitor triapine (3-AP) significantly reduces EwS growth in vitro and in vivo. Furthermore, we present evidence that pharmacological RRM2 inhibition by triapine can overcome chemoresistance against doxorubicin or gemcitabine, and synergize with cell cycle checkpoint inhibitors (CHEK1 or WEE1). Conclusions: Based on the aggressive phenotype mediated by and the druggability of RRM2 our results provide a translational rationale for exploiting RRM2 as a novel therapeutic target in EwS and prompt further clinical investigations.

Sign in / Sign up

Export Citation Format

Share Document