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PubMed2003

Treatment of Ewing sarcoma family of tumors: current status and outlook for the future.

BACKGROUND: The Ewing sarcoma family of tumors (ESFT) comprises a group of well-characterized neoplasms with aggressive behavior. Despite significant progress with the use of intensive multiagent chemotherapy and local control measures, a significant proportion of patients die of disease progression. Chemotherapy dose intensification and autologous hematopoietic stem cell transplant (HSCT) have been explored by many institutions without obvious benefit in high-risk patients. Our current understanding in the biology and treatment of ESFT suggests that a more rational approach to the development of risk-adapted therapy should be undertaken. PROCEDURE: We performed a review of the most relevant data regarding the current status in the treatment of ESFT. The results of the major American and European cooperative groups were analyzed, including the treatment strategies used and the prognostic factors identified for both localized and metastatic ESFT. RESULTS: The intensification of alkylating agents and topoisomerase-II inhibitors is feasible and has resulted in some survival improvement for selected patients. This benefit seems to be restricted to patients with localized disease, and a proportion of survivors are at risk of developing treatment-related hematologic malignancies. Nevertheless, these advances have resulted in a re-definition of prognostic factors, which may help to define risk groups based on tumor load parameters as well as biologic factors (type of fusion transcript and histologic response to chemotherapy). Patients with advanced metastatic disease may benefit from HSCT. New strategies such as immunotherapy and the use of biologic modifiers may have a role in the treatment of ESFT. CONCLUSIONS: Future treatment for ESFT should consider risk-adapted strategies and the inclusion of newer therapies such as biologic modifiers for the minimal residual disease. A modified risk-adapted therapy is proposed.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1981

Collateral venous flow through uterus caused by retroperitoneal extraosseous Ewing sarcoma.

We report on a twenty-four-year-old woman with extraosseous Ewing sarcoma in the right retroperitoneum. The tumor blocked the common iliac vein, and because of an intervening pregnancy, the blood flow was shunted through the uterus and left ovarian vein back into the vena cava. She died within nine months despite radical surgery, radiotherapy, and chemotherapy.

Adult↗

PubMed2003

The epidermal growth factor receptor HER2 is not a major therapeutic target in Ewing sarcoma.

BACKGROUND: Although chimeric EWS gene and Ets gene fusions are pathognomonic of Ewing sarcoma (ES) and primitive neuroectodermal tumors (PNET), the molecular pathogenesis of these pediatric malignancies is poorly understood. Recently, the human epidermal growth factor (HER)-2 receptor, which plays an important role in the biology of certain epithelial cancers, has been implicated in ES tumor cell line growth and chemosensitivity. MATERIALS: To investigate whether HER2 might be a rational target for ES/PNET therapy, five ES cell lines and 13 archival primary ES/PNET samples were examined by immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH) for evidence of HER2 overexpression. RESULTS: Although several ES cell lines demonstrated modest constitutive HER2 expression by immunoblot, none of the ES cell lines or primary tumor samples showed evidence of HER2 overexpression by IHC or HER2 gene amplification by FISH. Moreover, treatment of human ES cell lines with the HER2-targeted agent trastuzumab (Herceptin) had little effect on cell survival, proliferation, or growth in semi-solid medium. CONCLUSIONS: These results suggest that HER2 is not a biologically or therapeutically important pathway in ES/PNET.

Antibodies, Monoclonal↗

PubMedDate not supplied

Ki-1+ large-cell anaplastic lymphoma after Ewing sarcoma.

PURPOSE: A large cell anaplastic lymphoma that developed after treatment of a Ewing sarcoma (ES) is described. PATIENT: An 11-year-old girl with a pelvic ES developed a large cell, Ki-1+, anaplastic lymphoma in the same anatomic location 10 months after multimodal therapy. RESULTS: ES recurred in the primary site 16 months after allogeneic marrow transplantation and 3.5 years after initial diagnosis, but the patient remains in remission from her lymphoma. CONCLUSION: The occurrence of lymphoma and ES in a short time interval in the same patient is very unusual. Whether etiologic factors other than chemoradiotherapy, including genetic disposition, play a role remains to be elucidated.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1996

Unusual presentation of Ewing sarcoma with t(21;22) in a 3-year-old boy.

PURPOSE: We describe a 3-year-old boy with widespread, metastatic Ewing sarcoma and an unusual translocation, involving chromosomes 21 and 22. MATERIALS AND METHODS: Cytogenetic studies were performed on a biopsy of the primary tumor. These included GTG banding and fluorescence in situ hybridization. RESULTS: A balanced translocation between chromosomes 21 and 22 was noted with translocation breakpoints at bands 21q22 and 22q12. CONCLUSIONS: The t(21;22) translocation represents a new cytogenetic abnormality that may be associated with Ewing sarcoma. Its prognostic significance, if any, remains to be determined.

Child, Preschool↗

PubMed2001

Acquisition of secondary structural chromosomal changes in pediatric ewing sarcoma is a probable prognostic factor for tumor response and clinical outcome.

BACKGROUND: The Ewing sarcoma (ES) group of tumors commonly have the t(11;22)(q24;q12) or other rearrangements involving 22q12. In addition to these consistent aberrations, both numeric and structural aberrations have been reported: namely gains of chromosomes 8 and 12, the unbalanced translocation t(1;16), and deletions at the short arm of chromosome 1. METHODS: To evaluate the frequency and to study the prognostic implications of some of these aberrations in children, the authors performed a pilot study of 26 ES pediatric patients by classic cytogenetics and/or interphase fluorescence in situ hybridization (FISH) and compared these data with clinical parameters. RESULTS: Gains of chromosomes 8 and 12 were detected, by interphase FISH, in 48% (10 of 21) and 38% (6 of 16) of the tumors, respectively, and this was not significant with respect to treatment response. Statistical analysis revealed that the presence of additional secondary structural chromosomal aberrations was associated with an unfavorable outcome (P = 0.0034 as an independent prognostic value as an unfavorable marker). Presence of metastasis at diagnosis also was found to be associated with poor outcome (P = 0.0131). Spectral karyotyping analysis was shown to facilitate the detection of more complex structural chromosomal aberrations in a representative ES tumor. CONCLUSIONS: It is important to determine whether additional structural chromosomal aberrations are present in ES tumors because it appears that a more complex karyotype with multiple chromosomal aberrations is associated with poor outcome in ES.

Adolescent↗

PubMed2005

Definitive surgery and multiagent systemic therapy for patients with localized Ewing sarcoma family of tumors: local outcome and prognostic factors.

BACKGROUND: The local management of Ewing sarcoma family of tumors (ESFT) often centers on the surgical resectability of the primary lesion and physician biases regarding differences in the morbidity between primary surgical and radiotherapeutic management. METHODS: The authors retrospectively reviewed the records of 33 patients with localized ESFT who underwent surgery and received systemic chemotherapy at St. Jude Children's Research Hospital (Memphis, TN). Two multiagent systemic chemotherapy regimens were used: 14 patients received vincristine, doxorubicin, cyclophosphamide, and actinomycin D (VACA), and 19 received VACA in combination with ifosphamide and etoposide. The primary tumor was surgically resected via a wide, local excision (n = 32) or a marginal excision (n = 1)performed either at diagnosis or after 3-5 months of systemic chemotherapy. Clinical outcome and prognostic factors for disease control were reported in the current study. RESULTS: The median follow-up for patients was 9.9 years. The 5-year and 10-year survival rates were 84.5% and 75.8%, respectively. At 5 years, the cumulative incidence of local disease recurrence was 12.5%, and the event-free survival (EFS) rate was 71.7%. The same values were found at 10 years. The site of tumor origin was a significant predictor of EFS. The survival rate of patients whose tumors arose in bone was 78.6%, and the survival rate of patients whose tumors originated in soft tissue was 25.0% (P = 0.028). No other factors investigated were predictive of outcome. CONCLUSIONS: Local disease control and overall outcome for patients with ESFT managed by multiagent systemic therapy and surgery was excellent. Local disease control rates remained near 90% at 10-year follow-up. Patients with extraosseous primary sites of disease may fare less well with this approach to therapy.

Adolescent↗

PubMed1985

Radiotherapy of non-metastatic ewing sarcoma.

Comparing the radiotherapy data of two groups of patients with non-metastatic Ewing sarcoma, we came to the following conclusions: intensive chemotherapy does not substitute for effective radiotherapy. Irradiation fields should be large enough to include the whole affected bone and the entire extra-osseous compartments, exempting only uninvolved epiphyses. Marginal recurrences are a major risk of the shrinking field technique. Single doses per fraction should be high and the total dose raised to the point of maximal irradiation tolerance of normal tissues.

Adolescent↗

PubMed1998

Reconstruction of the first and second metatarsals with free microvascular fibular bone graft after resection of a Ewing sarcoma.

We report the case of a 12-year-old girl with a Ewing sarcoma originating from the first metatarsal of one foot. Because the girl and her parents refused amputation of the foot, we resorted to complete local resection and to reconstruction of the first and second metatarsals with a free microvascular fibular bone graft. At 3 1/2 years after surgery, the patient continues to walk without a limp, and the mechanical function of the forefoot has been largely restored, although considerable loss of function of the big toe is apparent. Details of the surgical technique and tumor management are described.

Bone Neoplasms↗

PubMed1981

[Growth stimulation in a Ewing sarcoma after "macrophage blockade" in athymic (nude) mice].

Inoculation of cell suspensions of a Ewing sarcoma (1.5 to 2.0 x 10(8) viable cells/mouse) into thymus less nude mice bred under conventional conditions gave a 90% take rate after subcutaneous injection and 35% after intraperitoneal. Intraperitoneal take rate is raised to 90% by intraperitoneal pretreatment with India ink. The same pretreatment shortens the tumor doubling time after subcutaneous inoculation. Both events are probably caused by inhibition of macrophages and/or natural killer cells.

Adolescent↗

PubMed2004

Definitive irradiation in multidisciplinary management of localized Ewing sarcoma family of tumors in pediatric patients: outcome and prognostic factors.

PURPOSE: To assess the effect of radiation dose on local tumor control of the Ewing sarcoma family of tumors in 79 patients with localized disease treated at a single institution. METHODS AND MATERIALS: Thirty-seven patients received vincristine, actinomycin D, cyclophosphamide, and doxorubicin, and 42 received vincristine, actinomycin D, and cyclophosphamide, with alternating cycles of ifosfamide and etoposide; all underwent definitive radiotherapy (median dose, 37.5 Gy) with either low-dose (<40 Gy) or standard dose (> or =40 Gy) radiation delivered according to the protocol. We calculated the cumulative incidence of local treatment failure, disease recurrence, and overall survival and analyzed the effect of known prognostic factors and radiation dose. RESULTS: The cumulative incidence of local treatment failure at 10 years was 30.4% and that of disease recurrence was 40%. The overall survival rate was 64.5%. Patient age > or =14 years and tumor size > or =8 cm were adverse prognostic factors for local treatment failure; patient age > or =14 years was also associated with worse survival. Although the radiation dose alone did not predict for local treatment failure, the cumulative incidence of local failure at 10 years was 19% when tumors <8 cm were treated with <40 Gy, and no patient treated with standard doses (> or =40 Gy) developed local recurrence (p = 0.084). CONCLUSION: Tumor size and patient age predict for local tumor control in patients with Ewing sarcoma family of tumors treated with systemic therapy and definitive radiotherapy. Patients treated with reduced-dose radiotherapy experienced unacceptably high rates of local recurrence.

Adolescent↗

PubMed2003

Newly established Askin tumor cell line and overexpression of focal adhesion kinase in Ewing sarcoma family of tumors cell lines.

Askin tumor is a malignant small round cell tumor that originates from the thoracopulmonary region and is a member of Ewing sarcoma family of tumors (ESFT). Only a few Askin tumor cell lines have been established. An Askin tumor cell line, designated MP-ASKIN-SA, was established from the left thoracic tumor of a 13-year-old Japanese boy. ESFT is known to have a high rate of distant metastases at diagnosis. The genes controlling the spread of ESFT cells, however, have not been elucidated. G-banding chromosome analysis revealed that the MP-ASKIN-SA cell line has complex chromosomal abnormalities including trisomy 8. The EWS/FLI1 chimeric transcript and c-myc overexpression were revealed by the reverse transcriptase-polymerase chain reaction and Northern blot analysis. Furthermore, we investigated the expression of the focal adhesion kinase (FAK) gene in the ESFT cell lines using Northern blot analysis. In addition to the MP-ASKIN-SA cell line, six Ewing sarcoma cell lines, one peripheral nerve sheath tumor cell line, and two Askin tumor cell lines were analyzed. All ESFT cell lines, including MP-ASKIN-SA, expressed five- to twenty-eight-fold-increased values of FAK, as compared with fibroblasts obtained from the bone marrow of a healthy volunteer. These results raise the possibility that the overexpression of c-myc and FAK are involved in the poor prognosis of ESFT.

Adolescent↗

PubMed2005

Zoledronic acid inhibits primary bone tumor growth in Ewing sarcoma.

BACKGROUND: Zoledronic acid has been shown to be effective in the treatment of osteoporosis, hypercalcemia, and metastatic bone tumors. The efficacy of zoledronic acid on primary bone tumors has not been investigated. METHODS: A primary bone tumor mouse model was established. Intratibia injection of TC71 cells resulted in an osteolytic bone tumor. Four days after injection the mice were treated with zoledronic acid alone, paclitaxel alone, or zoledronic acid plus paclitaxel. Control mice were treated with phosphate-buffered saline. Bone tumor growth was assessed using a Faxitron Specimen Radiography System. The gene expression was detected by reverse-transcription polymerase chain reaction (RT-PCR), ELISA, and immunohistochemistry. Osteoclast formation was determined by tartrate-resistant acid phosphatase (TRAP) staining. RESULTS: Zoledronic acid induced apoptosis in TC71 human Ewing sarcoma cells and inhibited cell proliferation. Five weeks after injection, 89% of mice in the control group developed osteolytic bone tumors. Paclitaxel had little effect on bone tumor growth, with 78% of mice developing tumors. By contrast, 44% of mice treated with zoledronic acid developed bone tumors. The most effective treatment was zoledronic acid plus paclitaxel. Tumor incidence in the combination therapy group was only 22%. Osteoclasts were quantified using TRAP staining. There was a decrease in TRAP-positive osteoclasts in tumor tissues from zoledronic acid-treated animals compared to control animals. RT-PCR, immunohistochemistry, and ELISA assay demonstrated that zoledronic acid up-regulated osteoprotegerin expression. CONCLUSIONS: These results suggest that zoledronic acid induces apoptosis and inhibits primary bone tumor growth in Ewing sarcoma through a mechanism involving the up-regulation of osteoprotegerin. Zoledronic acid may provide a novel therapeutic approach for the treatment of patients with Ewing sarcoma.

Animals↗

PubMed2005

Favorable outcome of Ewing sarcoma family tumors to multiagent intensive preoperative chemotherapy: a single institution experience.

BACKGROUND: Aim of our study was to evaluate the efficacy of multiagent intensive preoperative chemotherapy in patients with Ewing sarcoma family tumors (ESFT), in order to succeed a better percentage of necrosis before surgical resection. PROCEDURE: Eighteen patients with ESFT were treated with the same multiagent intensive preoperative protocol. 5/18 patients had bone Ewings sarcoma (EWS) and 13/18 had peripheral primitive neuroectodermal tumor (PNET). None had metastases at diagnosis. Chemotherapy consisted of 5 or 6 cycles with vincristine, cisplatin, cyclophosphamide, and Adriamycin, followed by 12 cycles of vincristine, cyclophosphamide, and actinomycin-D. Five patients with EWS underwent total resection after 5-6 cycles of preoperative chemotherapy and prosthetic replacement was performed in two of them. In 3/13 patients with PNET the tumor was resected at diagnosis and in 1/13 after 5 cycles of chemotherapy, while 9/13 patients received chemotherapy only and/or radiotherapy. RESULTS: In patients with EWS, the histologic specimens of the resected tumors showed that tissue necrosis was 100% in four patients and 95% in one patient. The good histologic response reflects the effectiveness of this regimen in all ESFT. No patient had topical recurrence or developed metastatic disease during follow-up period (2-13 years, mean time 7.4 years). All patients had the scheduled cycles without delays or dose reductions. There were no major side effects of chemotherapy. CONCLUSIONS: The intensive chemotherapy schedule, comprising of 5-6 cycles preoperatively, seems to maximize the percentage of tumor necrosis, thus improving outcome. Our study implies that this combined therapy may improve the prognosis of ESFT.

Adolescent↗

PubMed2000

Induction of cell death by basic fibroblast growth factor in Ewing's sarcoma.

Ewing's sarcoma is thought to arise after developmental arrest of primitive neural cells during embryogenesis. Because basic fibroblast growth factor (bFGF) has a critical role in the regulation of cell survival, proliferation, and differentiation during embryogenesis, we have tested the hypothesis that bFGF and FGF receptors may contribute to the development of Ewing's sarcoma and may provide a mechanism for the modulation of their behavior. All four of the Ewing's sarcoma cell lines examined expressed bFGF and FGF receptors, which were detected by immunofluorescence and Western blotting. bFGF-induced a significant dose-dependent decrease in Ewing's sarcoma cell proliferation on plastic and reduced anchorage-independent growth in soft agar. Unexpectedly, this decrease in cell number reflected bFGF-induced apoptosis and necrosis, as demonstrated by electron microscopy, binding of annexin V, and staining with acridine orange. Induction of cell death was dependent on dosage of, and period of exposure to, bFGF. bFGF did not induce differentiation of Ewing's sarcoma cells in either the presence or the absence of serum or nerve growth factor. Treatment of NuNu mice with bFGF decreased growth of the highly tumorigenic Ewing's sarcoma cell lines. Histologically tumors grown in the NuNu mice treated with bFGF were less cellular than those in control mice, and showed an increased level of apoptotic nuclei. This is in contrast to the mitogenic effect bFGF has in most other cancer cells. In summary, bFGF decreases Ewing's sarcoma growth in vitro and in vivo by the induction of cell death. This novel observation may provide a new therapeutic strategy for Ewing's sarcomas.

Animals↗

PubMed1978

Metastatic Ewing sarcoma to the heart simulating adriamycin cardiotoxicity.

A 20-year-old man with metastatic Ewing Sarcoma developed severe congestive heart failure. Because he had been treated with a large amount of Adriamycin, the diagnosis was initially thought to be Adriamycin cardiotoxicity. However, ante- and post-mortem studies revealed the presence of massive cardiac metastases. At post-mortem, there was no evidence of Adriamycin cardiotoxicity. This case emphasizes that cardiac metastases must be considered in the differential diagnosis of heart failure in patients treated with Adriamycin.

Adult↗

PubMed2005

Concomitant Ewing sarcoma and acute lymphoblastic leukemia in a 5-year-old girl.

Malignancies from the Ewing family of tumors and acute lymphoblastic leukemia (ALL) are not known to be associated with each other. A 5-year-old girl was incidentally found to suffer from acute lymphoblastic leukemia during bone marrow staging for Ewing sarcoma of the radius. The simultaneous presence of two distinct neoplasms was confirmed by RT-PCR, with EWS/FLI1 type 1 rearrangement in the bone tumor and TEL/AML1 rearrangement in the marrow. She was treated with chemotherapy, radiotherapy, and surgery and was in remission of both diseases 31 months after diagnosis.

Child, Preschool↗

PubMed2002

Primary skeletal neoplasms: osteosarcoma and ewing sarcoma.

The care for children and adolescents with malignant bone tumors such as osteosarcoma and Ewing sarcoma will be improved by the magnetic resonance imager that can recognize the tumor early in its course and can stage it correctly for proper chemotherapy and local control. This can be accomplished by attention to the proper magnetic resonance imaging technique, recognition of the magnetic resonance appearance, and knowledge of the prognostic factors at diagnosis. Determination of response on follow-up static and dynamic contrast-enhanced magnetic resonance imaging and knowledge of optimal methods to determine recurrent tumor will improve the outcome for these children.

Adolescent↗