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PubMed1980

Intergroup Ewing's Sarcoma Study: local control related to radiation dose, volume, and site of primary lesion in Ewing's sarcoma.

One hundred ninety-three patients with localized Ewing's sarcoma treated at participating instutitions of the Intergroup Ewing's Sarcoma Study form the basis for this report. All patients received radiation therapy to the primary lesion and were randomized to receive vincristine, actinomycin-D, and cyclophosphamide (VAC) plus adriamycin (Regimen I); VAC alone (Regimen II); or VAC and bilateral pulmonary irradiation (Regimen III). Local control was achieved in 96% of the patients in Regimen I, and 86% of the patients in both Regimens II and III. The median duration of follow up was 83 weeks and median survival time was 172 weeks. Incremental doses of irradiation did not result in significant changes in the rate of local control of primary lesions. The local control rate was the same (92%) for tumors treated by means of whole-bone irradiation or with at least 5 cm of free margin around the lesion. The local control rate decreased to 79% for lesions treated with less than 5-cm margin. Excellent control was obtained for lesions involving the skull or spine (100%), and distal bones (fibula, 96% and tibia, 91%). Less favorable control rates were noted for pelvic and humeral lesions (84% and 79%, respectively). Bilateral pulmonary irradiation for subclinical disease played a role in lowering the incidence of lung metastases from 38% to 20% for patients treated with VAC. Lung metastases were similarly decreased (10%) when adriamycin was added to VAC chemotherapy.

Bone Neoplasms↗
PubMed1980

Ultrastructure of one Ewing's sarcoma of bone with endothelial character and a comparative review of the vessels in 27 cases of typical Ewing's sarcoma.

An atypical variant of Ewing's sarcoma, located in the left hip of a nine-year-old girl, is discussed at optical, histochemical and electron microscopical level. The endothelial appearing cells seem to play a main role in its histogenesis. Tumoral cells of an undifferentiated blastemic nature show round nuclei and bright lucent cytoplasm, being organized in solid sheets or vascular-like profiles. Alkaline and acid phosphatases are very prominent in all tumoral cells, and some of them also show PAS positive material. Its ultrastructure demonstrates an active pinocytic capacity, cytoplasmic filaments and Weibel-Palade bodies. Simultaneously a review is performed on 27 cases of typical Ewing's sarcoma of bone in order to compare its vessels of a reactive nature with this tumor. A differential diagnosis with hemangioendothelioma primary to bone is established.

Acid Phosphatase↗
PubMed1991

MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration.

This study reports on the specific expression of the MIC2 gene, a pseudoautosomal gene located on the short arms of the X and Y chromosomes, on Ewing's sarcoma (ES) and peripheral primitive neuroectodermal tumor (pPNET) cells. The gene product, a cell membrane protein, is recognized by the newly established monoclonal antibody (MoAb) HBA-71 and the previously described MoAb 12E7 and RFB-1. Furthermore, the reaction pattern of the MIC2 antibodies, especially HBA-71, with normal tissues and a great number of benign and malignant tumors (70 different tumors, 199 tumor samples), as well as the correlation between the specific chromosomal aberrations, i.e., the t(11;22) and the del(22) and the expression of this antigen, are demonstrated. Both ES and pPNET cells express the MIC2 gene in very high amounts, which represents a highly selective and almost unique feature of these cells, making an assignment of these tumors in one entity even more likely. The MIC2 antibodies are of great value for clinical and research purposes.

12E7 Antigen↗
PubMed1998

A Southwest Oncology Group and Cancer and Leukemia Group B phase II study of doxorubicin, dacarbazine, ifosfamide, and mesna in adults with advanced osteosarcoma, Ewing's sarcoma, and rhabdomyosarcoma.

BACKGROUND: Ewing's sarcomas, osteosarcomas, and rhabdomyosarcomas are significantly more responsive to chemotherapy than other sarcomas. Adjuvant chemotherapy is used routinely based on data from randomized trials. Although a percentage of children with locally advanced or metastatic tumors remain curable, few data exist regarding the tumor's natural history or response and survival in adults. METHODS: This Phase II study evaluated doxorubicin, dacarbazine, ifosfamide, and mesna (MAID) in adults with inoperable or metastatic Ewing's sarcoma, rhabdomyosarcoma, or osteosarcoma. RESULTS: Between 1987-1991, 81 patients were entered; 69 patients were eligible. One patient died of neutropenic infection. Ten patients (14%) responded completely and 34 patients (49%) had a complete or partial response. Response rates were significantly higher for patients with Ewing's sarcoma and rhabdomyosarcoma than for those with osteosarcoma (77%, 64%, and 26%, respectively; P < 0.005). Although there were no significant differences in progression free survival by histology, survival for patients with Ewing's sarcoma was significantly longer than for patients with osteosarcoma (P = 0.004.) At the time of last follow-up, 7 patients (10%) were alive without progression: 3 with Ewing's sarcoma, 1 with osteosarcoma, and 3 with rhabdomyosarcoma. CONCLUSIONS: MAID chemotherapy is an active regimen in adults with advanced or metastatic Ewing's sarcoma and rhabdomyosarcoma. Although there was no direct comparison with a doxorubicin and cisplatin-based regimen, the response rate and survival in patients with osteosarcoma suggest that doxorubicin and cisplatin-based chemotherapy would remain the accepted initial chemotherapy regimen. For patients with rhabdomyosarcoma and Ewing's sarcoma, 10-20% of patients remained disease free at 5 years.

Adolescent↗
PubMed1964

EWING'S SARCOMA.

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Adolescent↗
PubMed2005

[Primary cutaneous extraskeletal Ewing's sarcoma].

BACKGROUND: Cutaneous extraskeletal Ewing's sarcoma is rare, being seen principally in children. We report a case of cutaneous sarcoma in the sole of the foot in a child. CASE REPORT: A 9-year-old child with no medical history of note was presenting a skin tumor for 3 months on the heel of the right foot. This tumor was burgeoning and painful and measured 3.5 cm in diameter; it was ulcerative at the surface and covered with a crust. Histological and immunohistochemical examinations confirmed the diagnosis of Ewing's sarcoma. Staging examinations proved negative and the patient underwent polychemotherapy, resulting in complete regression of the tumor. COMMENTS: Until 1998, 37 cases of cutaneous and subcutaneous Ewing's sarcoma were reported, being seen in 21 girls and 16 boys. Mean age at diagnosis was 15 years and mean tumor size was 3 cm (range: 1 to 12 cm). The tumors were observed throughout the body, being seen in the sole of the foot in 2 cases. Confirmation of the diagnosis was made by histological examination (malignant proliferation of small round cells in the dermis), immunohistochemical examination (CD99+) and cytogenetic analysis (translocation between chromosomes 22 and 11). The prognosis for cutaneous Ewing's sarcoma appears more favorable than that of Ewing's sarcoma in bone. Of the 37 patients treated, 7 had metastases and 2 presented relapse. Treatment for cutaneous Ewing's sarcoma, though not codified, consists of polychemotherapy associated with surgery and/or radiotherapy.

Age of Onset↗
PubMed2006

Expression profiling of EWS/FLI identifies NKX2.2 as a critical target gene in Ewing's sarcoma.

Our understanding of Ewing's sarcoma development mediated by the EWS/FLI fusion protein has been limited by a lack of knowledge regarding the tumor cell of origin. To circumvent this, we analyzed the function of EWS/FLI in Ewing's sarcoma itself. By combining retroviral-mediated RNA interference with reexpression studies, we show that ongoing EWS/FLI expression is required for the tumorigenic phenotype of Ewing's sarcoma. We used this system to define the full complement of EWS/FLI-regulated genes in Ewing's sarcoma. Functional analysis revealed that NKX2.2 is an EWS/FLI-regulated gene that is necessary for oncogenic transformation in this tumor. Thus, we developed a highly validated transcriptional profile for the EWS/FLI fusion protein and identified a critical target gene in Ewing's sarcoma development.

Animals↗