Ewing Sarcoma: An Eponym Window to History

Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, ML7015, OH 45229, USA.
Sarcoma 01/2011; 2011(1357-714X):457532. DOI: 10.1155/2011/457532
Source: PubMed

ABSTRACT Ewing sarcoma was named after James R. Ewing, an eminent American pathologist at Cornell who described the first cases in 1921. Although he is best remembered for this singular achievement, Ewing's contributions to the study of cancer were far more profound and influential. He essentially launched oncology as a discipline with the publication of his seminal textbook and founded the major American cancer societies that exist today. His vision of comprehensive cancer centers still drives our research infrastructure. Since his initial report, these organizations have helped us achieve numerous milestones in understanding and treating patients with Ewing sarcoma.

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    ABSTRACT: Background: Ewing sarcoma is the most common primary malignant tumour in patients younger than 10 years of age. The incidence is less than 1 per 1 million per year. Usually it is located in the diaphysis of long bones. Prognosis of these tumours has improved dramatically since the introduction of multi-agent chemotherapy, from an erstwhile 10% survival rate to the current 70% for patients with non-metastatic Ewing sarcoma. Method: A retrospective review of patients with histologically confirmed Ewing sarcoma who were treated in the Department of Orthopaedics, B.S. Medi-cal College during the time period from April 2000 to March 2012 was performed. Patients were divided into two groups: Group A included those treated by External Beam Radiotherapy (EBRT) + chemotherapy while Group B in-cluded the patients treated with surgery + chemotherapy. Results were analysed depending on the survival rates. Kap-lan-Meier survival curves were compared using log-rank test and a multivariate Cox proportional hazards model was calculated. Result: The survival curves of both the groups were not found to be significantly different. Conclusion: Treatment of Ewing tumour has multiple options. No one treatment modality is superior. Survival rates of patients treated by radiation + chemotherapy are not significantly different from those treated with surgery + chemotherapy.
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    ABSTRACT: Designation of a rare 'orphan' disease is usually conferred by a prevalence of one in 1,500 to 2,500 individuals. Increasingly, orphan diseases are also being defined by their molecular fingerprints. Rare diseases are uniquely challenging from a therapeutic standpoint; it is critical to modify clinical study design of treatments for orphan disorders as well as for the increasingly smaller molecular subsets within frequently occurring cancers. In spite of the immense challenges associated with developing a treatment for a rare disorder, some of the most groundbreaking therapeutic discoveries have been made in orphan malignancies. This situation may be because a limited number of driver molecular aberrations occur in rare disorders, which can be targeted by agents. Here, we describe drug-class examples of targeted therapies for orphan diseases, with particular emphasis on malignancies or tumour-prone nonmalignant conditions, as well as potential therapeutic strategies that can be adopted to treat these orphan conditions.
    Nature Reviews Clinical Oncology 09/2012; 9(11):631-42. DOI:10.1038/nrclinonc.2012.160 · 14.18 Impact Factor
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    ABSTRACT: Ewing sarcoma (ES) is rare in Japanese people, and only 30-40 patients develop the disease annually. To diagnose ES, molecular techniques that aim to detect characteristic fusion genes are commonly used in combination with conventional histological and immunohistochemical examinations. The treatment strategy for ES is characterized by multi-disciplinary collaboration between pediatric oncologists, medical oncologists, radiation oncologists, and orthopedic surgeons. In recent years, numerous large-scale national or international multi-institutional studies of ES have been performed. Pre- and postoperative intensive systemic chemotherapy with multiple anticancer drugs is the standard treatment method for ES. Depending on the obtained surgical margin, postoperative radiation might also be performed. If preoperative radiological examinations indicate that surgical excision would be difficult, preoperative radiation can be administered. As the treatment outcomes of ES have improved, late complications and secondary malignancies have become a problem. After treatment, patients with ES require very long-term follow-up in order to detect secondary malignancies and growth-related musculoskeletal complications.
    Journal of Orthopaedic Science 02/2015; 20(2). DOI:10.1007/s00776-014-0687-z · 0.94 Impact Factor
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