We report a response to pazopanib in a 69-year-old man with heavily pre-treated metastatic extraosseous Ewing sarcoma in addition to molecular profiling of his tumor. To our knowledge, this case is the earliest to demonstrate activity of an oral multi-targeted kinase inhibitor in Ewing sarcoma. This case provides rationale for adding a Ewing sarcoma arm to SARC024, a phase II study of regorafen...

BACKGROUND Ewing sarcoma family of tumors is the second most common primary bone tumor of childhood. Extraosseous Ewing sarcoma family of tumors is rare. We present a pediatric case of primary endobronchial Ewing sarcoma family of tumors. CASE REPORT A 12-year-old boy presented with dyspnea and chest radiography showed right pulmonary atelectasis. Chest computed tomography demonstrated tumor ...

There is a critical need in cancer therapeutics to identify targeted therapies that will improve outcomes and decrease toxicities compared to conventional, cytotoxic chemotherapy. Ewing sarcoma is a highly aggressive bone and soft tissue cancer that is caused by the EWS-FLI1 fusion protein. Although EWS-FLI1 is specific for cancer cells, and required for tumorigenesis, directly targeting this t...

Advances in knowledge: Lung metastases from Ewing sarcoma have traditionally been treated with whole-lung irradiation (WLI) and chemotherapy. WLI may unnecessarily treat uninvolved normal lung tissue and increase the risk of toxicity. Stereotactic body radiotherapy (SBRT) has proven to be a safe and effective treatment for primary lung cancer and lung metastases, but there is no significant pub...

A miRNA-based in vivo reporter deciphers cell heterogeneity Ewing sarcoma.

Ewing sarcoma is a primary bone tumor which was originally described by Lucke in 1866 but was defined in such detail by Sir James Ewing in 1921 that it is now associated with his name [1]. This tumor involves the spine primarily in 3.5% to 14.9% r ~ ~ases [2,3]. The review of 22 cases of Ewing Sarcoma of the spine by Pilepich et al in 1981 [4] remains the largest study of this pathology in such...

Ewing sarcoma is an aggressive, poorly differentiated neoplasm of solid bone that disproportionally afflicts the young. Despite intensive multi-modal therapy and valiant efforts, 70% of patients with relapsed and metastatic Ewing sarcoma will succumb to their disease. The persistent failure to improve overall survival for this subset of patients highlights the urgent need for rapid translation ...

MicroRNAs (miRs) have been identified as potent regulators of both normal development and the hallmarks of cancer. Targeting of microRNAs has been shown to have preclinical promise, and select miR-based therapies are now in clinical trials. Ewing Sarcoma is a biologically aggressive pediatric cancer with little change in clinical outcomes despite improved chemotherapeutic regimens. There is a s...

PURPOSE A low mutation rate seems to be a general feature of pediatric cancers, in particular in oncofusion gene-driven tumors. Genetically, Ewing sarcoma is defined by balanced chromosomal EWS/ETS translocations, which give rise to oncogenic chimeric proteins (EWS-ETS). Other contributing somatic mutations involved in disease development have only been observed at low frequency. EXPERIMENTAL...

Extraskeletal Ewing’s Sarcoma is a rare soft tissue tumor that is morphologically indistinguishable from the more common Ewing’s sarcoma of the bone. Primitive Ewing’s sarcoma of the breast is exceptionally reported. We present the case of a 26-year-old woman with Ewing sarcoma/PNET diagnosed by molecular biology showing the specific t ranscr ipt of Ewing/ per ipheral pr imi t ive neuroectoderm...