Osteosarcoma With Apparent Ewing Sarcoma Gene Rearrangement

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)

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Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Stomach

Case Reports in Oncology ◽

10.1159/000449126 ◽

2016 ◽

Vol 9 (3) ◽

pp. 666-671 ◽

Cited By ~ 4

Author(s):

Safi Khuri ◽

Hayim Gilshtein ◽

Sa’d Sayidaa ◽

Bishara Bishara ◽

Yoram Kluger

Keyword(s):

Ewing Sarcoma ◽

Primitive Neuroectodermal Tumor ◽

Gene Rearrangement ◽

Neoadjuvant Treatment ◽

Neuroectodermal Tumor ◽

Surgical Ward ◽

Healthy Female ◽

Lesser Curvature ◽

Upper Gastrointestinal

Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation). These findings were compatible with gastric ES/PNET. The fluorescence in situ hybridization analysis result for the EWSR1 gene rearrangement (11: 22 translocation) was positive. The patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found. The mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon. Total gastrectomy, distal pancreatectomy, splenectomy, and left adrenalectomy were done. The patient refused adjuvant treatment. She is free of disease 3 years after surgery.

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Targeting the p53 Pathway in Ewing Sarcoma

Sarcoma ◽

10.1155/2011/746939 ◽

2011 ◽

Vol 2011 ◽

pp. 1-17 ◽

Cited By ~ 22

Author(s):

Paul M. Neilsen ◽

Kathleen I. Pishas ◽

David F. Callen ◽

David M. Thomas

Keyword(s):

Cell Cycle ◽

Dna Damage ◽

Ewing Sarcoma ◽

Gene Rearrangement ◽

Cell Cycle Checkpoints ◽

Wild Type ◽

Damage Cell ◽

Downstream Signaling ◽

Functional Capabilities ◽

Wild Type P53

The p53 tumour suppressor plays a pivotal role in the prevention of oncogenic transformation. Cancers frequently evade the potent antitumour surveillance mechanisms of p53 through mutation of theTP53gene, with approximately 50% of all human malignancies expressing dysfunctional, mutated p53 proteins. Interestingly, genetic lesions in theTP53gene are only observed in 10% of Ewing Sarcomas, with the majority of these sarcomas expressing a functional wild-type p53. In addition, the p53 downstream signaling pathways and DNA-damage cell cycle checkpoints remain functionally intact in these sarcomas. This paper summarizes recent insights into the functional capabilities and regulation of p53 in Ewing Sarcoma, with a particular focus on the cross-talk between p53 and the EWS-FLI1 gene rearrangement frequently associated with this disease. The development of several activators of p53 is discussed, with recent evidence demonstrating the potential of small molecule p53 activators as a promising systemic therapeutic approach for the treatment of Ewing Sarcomas with wild-type p53.

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Small cell osteosarcoma with Ewing sarcoma breakpoint region 1 gene rearrangement detected by interphase fluorescence in situ hybridization

Annals of Diagnostic Pathology ◽

10.1016/j.anndiagpath.2012.08.004 ◽

2013 ◽

Vol 17 (4) ◽

pp. 377-382 ◽

Cited By ~ 9

Author(s):

Ema Dragoescu ◽

Colleen Jackson-Cook ◽

Gregory Domson ◽

Davis Massey ◽

William C. Foster

Keyword(s):

In Situ Hybridization ◽

Fluorescence In Situ Hybridization ◽

Ewing Sarcoma ◽

Gene Rearrangement ◽

Small Cell ◽

Breakpoint Region ◽

Small Cell Osteosarcoma

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Pediatric Tumors: Extraskeletal Ewing Sarcoma

10.2310/7800.16120 ◽

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)

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