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Ewing's sarcoma family of tumors: current management.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Bernstein, Mark L. Kovar, Heinrich Paulussen, Michael Randall, Robert Lor Schuck, Andreas Teot, Lisa A. Juergens, Herbert |
| Copyright Year | 2006 |
| Abstract | Ewing's sarcoma is the second most frequent primary bone cancer, with approximately 225 new cases diagnosed each year in patients less than 20 years of age in North America. It is one of the pediatric small round blue cell tumors, characterized by strong membrane expression of CD99 in a chain-mail pattern and negativity for lymphoid (CD45), rhabdomyosarcoma (myogenin, desmin, actin) and neuroblastoma (neurofilament protein) markers. Pathognomonic translocations involving the ews gene on chromosome 22 and an ets-type gene, most commonly the fli1 gene on chromosome 11, are implicated in the great majority of cases. Clinical presentation is usually dominated by local bone pain and a mass. Imaging reveals a technetium pyrophosphate avid lesion that, on plain radiograph, is destructive, diaphyseal and classically causes layered periosteal calcification. Magnetic resonance best defines the extent of the lesion. Biopsy should be undertaken by an experienced orthopedic oncologist. Approximately three quarters of patients have initially localized disease. About two thirds survive disease-free. Management, preferably at a specialist center with a multi-disciplinary team, includes both local control-either surgery, radiation or a combination-and systemic chemotherapy. Chemotherapy includes cyclic combinations, incorporating vincristine, doxorubicin, cyclophosphamide, etoposide, ifosfamide and occasionally actinomycin D. Topotecan in combination with cyclophosphamide has shown preliminary activity. Patients with initially metastatic disease fare less well, with about one quarter surviving. Studies incorporating intensive therapy followed by stem cell infusion show no clear benefit. New approaches include anti-angiogenic therapy, particularly since vascular endothelial growth factor is an apparent downstream target of the ews-fli1 oncogene. |
| Starting Page | 630 |
| Ending Page | 630 |
| Page Count | 1 |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://intl-theoncologist.alphamedpress.org/content/11/5/503.full.pdf |
| Alternate Webpage(s) | http://theoncologist.alphamedpress.org/content/11/5/503.full.pdf |
| PubMed reference number | 16720851v1 |
| Volume Number | 11 |
| Issue Number | 5 |
| Journal | The oncologist |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | Actinomycin Adverse reaction to drug Angiogenic Process Bone Marrow Bone Sarcoma Bone Tissue Bone pain Calcinosis Chromosomal translocation Chromosomes, Human, Pair 11 Chromosomes, Human, Pair 22 Conflict (Psychology) Cyclophosphamide Dactinomycin Disease-Free Survival Doxorubicin Ehlers-Danlos Syndrome Endothelial Growth Factors Etoposide Ewings sarcoma Ewings sarcoma-primitive neuroectodermal tumor (PNET) FLI1 Transcription Factor Growth Factor Ifosfamide Kaposi Sarcoma Malignant Bone Neoplasm Neoplasm Metastasis Neoplasms Neuroectodermal Tumors Neurofilament Proteins Numerous Oncogenes Orthopedics Parkinson Disease Patients Plain x-ray Rhabdomyosarcoma Stem cells Systemic Chemotherapy Technetium TC-99M Pyrophosphate Tissue membrane Topotecan Vincristine Widespread Disease anatomical layer localized disease lung metastases soft tissue |
| Content Type | Text |
| Resource Type | Article |
