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PubMed2003

Molecular cytogenetic parameters in Ewing sarcoma.

To evaluate possible genomic instability and possible random aneuploidy, we applied comparative genomic hybridization and fluorescence in situ techniques, and evaluated telomerase activity in 16 cases of Ewing sarcoma (EWS) and compared the results to 7 controls. Common secondary aberrations (gains of chromosomes 8 and 12) were found in the study group. There was a direct correlation between the detection of random aneuploidy and development of tumor relapse (P = 0.0047). Other detectable abnormal parameters (secondary) and high telomerase activity were also more common among the cases with relapse but did not reach a statistical significance (probably because of the small sample size). In EWS, the detection of random aneuploidy seems to be a sensitive parameter in the prediction of tumor relapse.

Adolescent↗

PubMed2001

Selective use of whole-lung irradiation for patients with Ewing sarcoma family tumors and pulmonary metastases at the time of diagnosis.

PURPOSE: The benefit of whole-lung irradiation (WLI) for patients who have pulmonary metastases (PM) of Ewing sarcoma family tumors (ESFT) is unclear. At our institution, WLI is reserved for patients with PM that do not respond completely to induction chemotherapy. We reviewed our experience to assess the impact of WLI on clinical outcome. PATIENTS AND METHODS: Twenty-eight patients with ESFT and PM were treated in three consecutive institutional trials (1979-1996). Extent of pulmonary involvement at diagnosis, response of PM after induction chemotherapy, local treatment of PM thereafter, and clinical outcome were recorded. Treatment included primary tumor surgery and/or radiotherapy and 42 to 58 weeks of multiagent chemotherapy. RESULTS: Only eight patients (29%) received WLI. For the entire study group, the estimated 5-year event-free survival was 22.9% +/- 9.0%; the 5-year survival was 37.3% +/- 9.8%. Complete resolution of PM after induction chemotherapy was not correlated with survival (P = 0.53), nor was treatment with WLI (P = 0.87). CONCLUSIONS: The comparable survival of patients with poor and good response of PM to induction chemotherapy suggests that WLI may benefit poor responders. The use of WLI in good responders may provide similar benefit and merits further study.

Adolescent↗

PubMed2004

[Ewing's sarcoma].

Ewing's sarcomas account for 6.8% of all primary malignant bone tumors and are probably a neurogenic, undifferentiated, high-grade malignancy, which usually affects the bones of children 5-15 years of age. Pain and swelling are the most common symptoms. Increase of CRP and erythrocyte sedimentation rate, leucocytosis, and anemia are frequently seen. Radiologically, they show permeative bone destruction on plain radiographs. When arising in the diaphysis of long bones, laminated, "onion-skin" periosteal reaction is seen. The tumor shows muscle density on CT, iso-signal intensity on T1-weighted MR images, and high signal intensity on T2-weighted MR images. Intramedullary invasion and skip lesions can be detected on MR images. Histologically, the tumor is uniformly composed of sheets of small round cells closely packed and without any matrix product. Glycogen granules are demonstrated in the cytoplasm by periodic acid-Schiff (PAS) and diastase reactions. Immunohistochemically, Ewing's sarcomas are positive for vimentin and MIC-2 gene product (CD99). Reciprocal translocation, i.e., t(11;22) (q24;q12), is seen in the tumor cells. EWS/FLI-1 fusion gene can be demonstrated, which can be a complementary method in diagnosing this tumor. Because Ewing's sarcomas are chemosensitive and radiosensitive, they are treated by a combination of chemotherapy, surgery, and radiotherapy. Neoadjuvant chemotherapy consists of preoperative chemotherapy and postoperative chemotherapy. Preoperative chemotherapy aims at eradicating distant micrometastasis, reducing the primary tumor volume, and evaluating the efficacy of the chemotherapeutic agents. Surgery is performed as a local treatment by excising the tumor using the wide procedure. If surgery is impractical, curative radiotherapy is performed instead of excision. When surgery is performed without complete wide procedure, adjuvant radiotherapy is carried out to eradicate the residual tumor cells. Postoperative chemotherapy aims to eradicate the distant micrometastasis. Recently, myeloablative, high-dose chemotherapy followed by autologous bone marrow transplantation is being attempted for poor-prognosis patients and good results have been reported.

Adult↗

PubMed1987

Prognostic factors in the treatment of Ewing's sarcoma. The Ewing's Sarcoma Study Group of the German Society of Paediatric Oncology CESS 81.

From 1981 up to February 1985, a total of 93 protocol patients entered the study CESS 81. The protocol recommended an initial 18-week period of polychemotherapy (VACA) followed by local therapy and two additional cycles of chemotherapy. Local therapy consisted either of radical surgery or of incomplete resection plus postoperative irradiation with 36 Gy or of radiotherapy alone (46 and 60 Gy). Centrally located lesions were always irradiated with 60 Gy. This article summarizes the data after 5 years. Data of 93 patients were analysed in October 1986 after a median follow-up of 37 months. The projected 5-year survival is 50%. The relapse rate was 42%, distant relapses occurred in 19%, local (plus distant) relapses in 23%. Most of the relapses occurred during the first 3 years of observation. Failure rate was high in patients undergoing irradiation alone (44%). Initial tumour mass (greater than 100 ml) and histopathologic response to initial chemotherapy were identified as major prognostic factors. Tumour site and type of local therapy were not significant if patients were categorised by tumour volume. In small lesions, surgery and radiotherapy were equally effective. In large lesions greater than 100 ml volume, a trend towards a better prognosis in surgically treated patients was observed. The results of CESS 81 emphasize the importance of permanent local control in Ewing's sarcoma even in the presence of systemic control by an effective multi-drug chemotherapy.

Adolescent↗

PubMed2001

Ewing sarcoma and sinonasal neuroectodermal tumors as second malignant tumors after retinoblastoma and other neoplasms.

BACKGROUND: Excesses of various childhood cancers have been reported after retinoblastoma, including a trickle of Ewing sarcoma (ES) and perhaps histologically similar olfactory neuroblastoma, both of which are neural tumors. To update and advance this information, case reports were sought by an extensive review of the literature. PROCEDURE: The search was made through the use of PubMed, and the Web of Science (Citation Index Expanded), keying on primary references. Three sinonasal cancers diagnosed as ES were immunohistochemically stained for MIC-2 protein (positive in ES). RESULTS: Retinoblastoma occurred before ES in ten cases (seven bilateral). In four others, retinoblastoma (three bilateral) developed before sinonasal neural tumors (poorly differentiated). ES also occurred after 14 cancers other than retinoblastoma (five lymphomas, four leukemias, and one each of five miscellaneous cancers). The predominance of retinoblastoma prior to ES differs markedly from the low-frequency of retinoblastoma among childhood cancers in the general population. On the contrary, cancers other than retinoblastoma were proportionate to those in the general population. Previously, retinoblastoma followed by excesses of osteosarcoma and soft tissue sarcomas has been attributed to the action of the inherited RB-1 gene. The sinonasal tumors stained negative for MIC-2 protein. CONCLUSIONS: Heritable retinoblastoma may predispose to ES and perhaps to a subset of poorly differentiated neuroectodermal tumors in the sinonasal region that may be related to olfactory neuroblastoma.

Adolescent↗

PubMed1993

Ewing sarcoma 11;22 translocation produces a chimeric transcription factor that requires the DNA-binding domain encoded by FLI1 for transformation.

The 11;22 chromosomal translocation specifically linked to Ewing sarcoma and primitive neuroectodermal tumor results in a chimeric molecule fusing the amino-terminal-encoding portion of the EWS gene to the carboxyl-terminal DNA-binding domain encoded by the FLI1 gene. We have isolated a fourth EWS-FLI1 fusion cDNA that is structurally distinct from the three forms previously described. To determine the transforming activity of this gene, alternative forms of the EWS-FLI1 fusion were transduced into NIH 3T3 cells. Cells expressing either type 1 or type 4 fusion constructs formed foci in culture and colonies in soft agar, indicating that EWS-FLI1 is a transforming gene. EWS-FLI1 deletion mutants were created to map functionally the critical regions within the chimera. Deletion of either the EWS domain or the FLI1 corresponding to the DNA-binding domain totally abrogated the ability for EWS-FLI1 to transform 3T3 cells. These data indicate that the oncogenic effect of the 11;22 translocation is caused by the formation of a chimeric transcription factor. Formation of chimeric transcription factors has now been demonstrated to promote tumors of both neuroectodermal and hematopoietic origin, suggesting that this may be a common mechanism in human carcinogenesis.

Amino Acid Sequence↗

PubMed1995

Massive allografts in the treatment of osteosarcoma and Ewing sarcoma in children and adolescents.

A retrospective review was performed of the results of all allograft reconstructions that had been done after the resection of an osteosarcoma or an Ewing sarcoma in a skeletally immature patient between 1982 and 1989 at The Hospital for Sick Children in Toronto. There were twenty-six patients. Six reconstructions were intercalary, sixteen were resection arthrodeses, three followed resection of a bone segment including the epiphysis (osteoarticular reconstruction), and one was a replacement of the entire humerus. Resection arthrodesis about the knee was performed with a smooth intramedullary rod and with one growth plate left intact. Six procedures were performed in the upper extremity. Excluding the patients who died, the average duration of follow-up was five years and three months. Twenty-one of the twenty-six patients had reached skeletal maturity at the time of follow-up. Eighteen (69 per cent) of the patients had a good or excellent result, four (15 per cent) had a fair result, and four had a failure. Twenty patients (77 per cent) had at least one complication (other than a limb-length discrepancy), and fourteen (54 per cent) sustained at least one fracture of the allograft. Fifteen patients who had had a reconstruction in the lower extremity had survived with survival of the allograft at the time of the latest follow-up. A limb-length discrepancy of at least two centimeters developed in nine of the fifteen patients. Five were managed with a contralateral epiphyseodesis, and one of them had an unsuccessful attempt at limb-lengthening as well. The patients who had a limb-length discrepancy of more than three centimeters at the time of follow-up had been significantly younger (p < 0.05) at the time of the reconstruction than those who had a smaller discrepancy. Three allografts (12 per cent), two of which were implanted early in the series, became infected. Soft-tissue coverage is of paramount importance for the prevention of infection, and we now routinely perform primary muscle (gastrocnemius or latissimus dorsi) transfers when dealing with an inadequate muscle envelope. Twelve patients were followed for more than four years (average, six years and seven months); they had no complications other than increased limb-length discrepancy and one subluxation of the shoulder after the first four years following the reconstruction. Although the rate of complications is higher than in adults, allograft reconstruction remains a useful option for the management of skeletally immature individuals.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

PubMed2006

Central nervous system extraosseous Ewing sarcoma: radiologic manifestations of this newly defined pathologic entity.

Although these entities are histologically similar, recent advances in molecular genetics have allowed the distinction of central nervous system extraosseous Ewing sarcoma (CNS-EES) from central primitive neuroectodermal tumors (c-PNET) including medulloblastoma and supratentorial PNET. We present 2 cases of pathologically confirmed CNS-EES. Knowledge of CNS-EES as a distinct entity enables the neuroradiologist to suggest the proper diagnosis and the need for special immuno-histochemical and molecular studies to confirm the diagnosis. Because treatment and prognosis are vastly different, the proper diagnosis of CNS-EES versus c-PNET is critical.

Brain Neoplasms↗

PubMed1994

MR of primary extraosseous Ewing sarcoma.

We present MR imaging findings of a 15-year-old girl with a history of chronic back pain, proved by biopsy to be caused by primary extraosseous Ewing sarcoma, extending into the spinal epidural space. We also demonstrate the involvement of the adjacent structures and encroachment on the intervertebral foramina.

Adolescent↗

PubMed1998

Primary metastatic (stage IV) Ewing tumor: survival analysis of 171 patients from the EICESS studies. European Intergroup Cooperative Ewing Sarcoma Studies.

BACKGROUND: In the multicenter European Intergroup Cooperative Ewing's Sarcoma Studies, localized Ewing tumors of bone were treated by combination chemotherapy with surgery and/or radiotherapy. Patients with primary metastases (pm-pts) were treated in high risk protocols. PATIENTS AND METHODS: One hundred seventy-seven pm-pts were registered from January 1990 to December 1995, 171 were evaluable for survival analyses. Thirty-six pm-pts received myeloablative megatherapy with stem cell rescue following conventional treatment. Bilateral whole lung irradiation (WLI) was administered in 57 pm-pts with pulmonary involvement. Event-free survival (EFS) rates were estimated by Kaplan-Meier analysis. Prognostic factors were identified by log-rank statistics, Cox procedures and logistic regression. RESULTS: Eighty-nine deaths were recorded by 1 February 1997, EFS four years after diagnosis for all 171 pm-pts was 0.27. EFS for isolated lung metastases was 0.34, for bone/bone marrow (BM) metastases, 0.28, and for combined lung plus bone/BM metastases, 0.14 (P < 0.005). WLI improved outcome in case of isolated pulmonary involvement (0.40 vs. 0.19, P < 0.05). In pm-pts with combined pulmonary/skeletal metastases, intensification by megatherapy and/or WLI improved EFS from 0.00 to 0.27 (P = 0.0001). CONCLUSIONS: EFS four years after diagnosis in patients with disseminated Ewing tumors is 0.27. Whole lung irradiation and megatherapy improve outcome in subgroups of patients with disseminated Ewing tumors is 0.27. Whole lung irradiation and megatherapy improve outcome in subgroups of patients with disseminated Ewing disease.

Adolescent↗

PubMedDate not supplied

Immunophenotypic profile and FISH analysis in a case of Ewing's sarcoma.

Ewing's sarcoma arising in the right fibula was diagnosed in a 12 years old female, considering clinical presentation, radiological findings and histological features. Our work points towards a possible neuroectodermal origin of Ewing's sarcoma, since MIC2/12E7 marker was found to be positive. Translocation t (11;22) (q24;q12) was found as the main cytogenetic change in this tumor, by means of FISH analysis.

12E7 Antigen↗

PubMed2005

Multiple cardiac metastases from Ewing's sarcoma.

Ewing's sarcoma commonly arises from bone and sometimes from extraskeletal soft tissues. It commonly metastasizes to the lungs, bone, bone marrow and brain. Cardiac metastasis is extremely uncommon. The present report discusses a case of Ewing's sarcoma metastasizing to the heart at the time of initial presentation.

Acetabulum↗

PubMed2000

Absent or reduced expression of the caspase 8 gene occurs frequently in neuroblastoma, but not commonly in Ewing sarcoma or rhabdomyosarcoma.

PROCEDURE: To clarify whether the caspase 8 gene is involved in the pathogenesis of neuroblastoma (NB), we examined alterations of the caspase 8 gene in 15 NB, seven Ewing sarcoma (ES), and eight rhabdomyosarcoma (RMS) cell lines, using reverse transcription-polymerase chain reaction (RT-PCR) and RT-PCR single-strand conformation polymorphism (SSCP) analyses. RESULTS: The caspase 8 gene was not expressed in 11 (73%) of 15 NB cell lines, it was absent in only one of seven ES cell lines, but was present in all eight RMS cell lines examined. No mutations were detected in any cell lines examined. CONCLUSIONS: Inactivation of the caspase 8 gene is considered to be involved in the pathogenesis of NB, but not ES or RMS.

Caspase 8↗

PubMed2001

Immunohistochemical detection of EWS and FLI-1 proteinss in Ewing sarcoma and primitive neuroectodermal tumors: comparative analysis with CD99 (MIC-2) expression.

The molecular analysis of the t(11;22) rearrangement involving EWS/FLI-1 genes is likely to be of diagnostic value in Ewing sarcoma (ES) and primitive neuroectodermal tumors (PNET). The objective of the current study was to analyze the immunohistochemical expression of the EWS and FLI-1 proteins in a group of small round-cell tumors (SRCT) to determine their specificity and relevance in their differential diagnosis. Forty-eight cases-10 conventional ES, 4 large-cell ES, 5 PNET, 9 neuroblastomas (NB), 6 undifferentiated synovial sarcomas (SS), 5 rhabdomyosarcomas (RB), 5 non-Hodgkin lymphomas (NHL), 1 round-cell liposarcoma, and 3 mesenchymal chondrosarcomas-were analyzed. Immunocytochemistry was performed on paraffin sections after the LSAB method and antigen retrieval using ethylenediaminetetraacetic acid buffer (pH 6). Primary antibodies included FLI-1 (C-19), EWS (N-18), EWS (C-19), and CD99 (MIC-2). As expected, CD-99 expression was found in 100% of ES/PNET cases, in 2 cases of RB, 2 SS, and 1 NHL. FLI-1 protein was observed as nuclear staining in 16 cases of ES/PNET (84%) and in 4 cases of NHL, 2 NB, and 3 SS. Normal endothelial cells and lymphocytes also were positive. EWS expression (both proteins N-18 and C-19) was detected not only in 95% of ES/PNET cases but also in more than 50% of cases from the other tumoral types (4 of 9 and 7 of 9 NB, 5 of 6 and 6 of 6 SS, 3 of 5 and 5 of 5 RB, and 2 of 5 and 3 of 5 NHL, respectively). Whereas EWS expression does not appear specific for ES/PNET, analysis of FLI-1 expression together with CD-99 is a powerful marker for ES/PNET and important factors in the differential diagnosis of SRCT.

12E7 Antigen↗

PubMed2001

Tumor volume or dynamic contrast-enhanced MRI for prediction of clinical outcome of Ewing sarcoma family of tumors.

BACKGROUND: The identification of risk factors that predict poor clinical outcome at the time of diagnosis could lead to intensified early therapy and improved outcome for pediatric patients with Ewing sarcoma family of tumors (ESFT). OBJECTIVE: To compare the effectiveness of static magnetic resonance (MR) imaging measurements of tumor volume with variables obtained by dynamic contrast-enhanced MR imaging (DEMRI) in predicting ESFT outcome. METHODS: MR examinations that included DEMRI were retrospectively reviewed. The analyses included 45 examinations of 21 patients with ESFT (performed from 1992 to 1996). Tumor volumes were measured on the static MR images, and the regions of interest were selected for DEMRI analysis. The relationships of static MR imaging and DEMRI variables with the probability of progression-free survival (PFS) and disease-free survival (DFS) were determined. RESULTS: Larger tumor volume at the time of diagnosis predicted poorer PFS and DFS estimates. No DEMRI variable predicted outcome. CONCLUSION: Determination of tumor volume by static MR imaging at the time of diagnosis is a simple and reliable method of predicting the clinical outcome of patients with ESFT. DEMRI is not as reliable a technique as static MR imaging for predicting the outcome of these patients.

Adolescent↗

PubMed2004

[Young adults with Ewing's sarcoma].

Ewing's sarcoma was diagnosed in three men, one aged 22 and two aged 30. The disease was diagnosed by biopsy and chromosome investigations (t(11;22)-translocation). In the youngest patient with localised disease, supplementary radiotherapy was withheld in view of the good results of induction chemotherapy, surgery and consolidation chemotherapy. However, four months later, there was a localised recurrence, again followed by induction chemotherapy, chemotherapy at high dosage, stem cell transplantation, radiotherapy and finally surgical intervention, after which a complete remission was achieved. The 30-year-old man with localised disease was given induction chemotherapy, surgery, consolidation chemotherapy and radiotherapy; 14 months after the diagnosis he was in good condition. The other 30-year-old man had metastases in TXII and both lungs. Despite intensive therapy he died 8 months after diagnosis. Ewing's sarcoma is a musculoskeletal malignancy that occurs in children and adolescents but also in young adults. It generally manifests itself as a painful swelling originating in bone or soft tissue. There are often accompanying symptoms such as weight loss and fever. In 20-25% of cases there are already metastases (to the lungs, bone and bone marrow) by the time of diagnosis. The diagnosis and treatment of this rare, therapy-sensitive disease should take place in a study setting and in co-operation with a multidisciplinary sarcoma working group.

Adult↗

PubMed2005

Ewing sarcomas with p53 mutation or p16/p14ARF homozygous deletion: a highly lethal subset associated with poor chemoresponse.

PURPOSE: EWS-FLI1 fusion type, p53 mutation, and homozygous deletion of p16/p14ARF have each been shown to be prognostically significant in Ewing sarcoma (ES). We provide the first combined prognostic analysis of these three molecular parameters in ES. PATIENTS AND METHODS: We studied 60 patients with ES (stage: localized in 54, metastatic in six). All cases were confirmed to contain the EWS-FLI1 (29 type 1, 12 type 2, 14 other types) or EWS-ERG fusions (five cases). Homozygous deletion of p16/p14ARF, and p53 mutations were determined by fluorescent in situ hybridization and Affymetrix (Santa Clara, CA) p53 GeneChip microarray hybridization, respectively. RESULTS: Eight cases (13.3%) contained point mutations of p53, and eight cases (13.3%) showed p16/p14ARF deletion, including one case with both alterations. Among 32 cases with data on histologic chemoresponse, all 10 with alterations in p53 or p16/p14ARF showed a poor chemoresponse (P = .03). Variables predicting poorer overall survival included p53 mutation alone (P < .001), either p53 or p16/p14ARF alteration (P < .001), and stage (P < .01). In multivariate analysis, alterations of p53 and/or p16/p14ARF as a single variable, was the most adverse prognostic factor (P < .001), followed by stage (P = .04). In a multivariate analysis with alterations of p53 and p16/p14ARF as separate variables, both were significant (P < .001 and P = .03, respectively). Six cases with p16/p14ARF deletion were also studied for co-deletion of the contiguous methylthioadenosine phosphorylase gene, and this was detected in four cases. CONCLUSION: Alterations in p53 or p16/p14ARF are found in a fourth of ES cases and define a subset with highly aggressive behavior and poor chemoresponse.

Adolescent↗

PubMed1999

Effect of low-dose radiation therapy when combined with surgical resection for Ewing sarcoma.

BACKGROUND: As an alternative to high-dose irradiation, limited surgery and low-dose irradiation have been investigated as a means to achieve local control. We retrospectively examined the clinical characteristics, treatment, and outcome for 25 patients with Ewing sarcoma treated with limited surgery and low-dose irradiation. PROCEDURE: The records of 25 patients (age 4-48 years) were reviewed who were treated between 1979 and 1996 at Memorial Sloan-Kettering Cancer Center. At the time of diagnosis, 21 of the 25 patients had prognostically unfavorable tumors including the presence of metastatic disease (n = 12), an axial primary (n = 17), and a tumor measuring greater than 8 cm (n = 18). The primary tumor was completely resected (wide local excision) in 13 patients, incompletely resected (marginal excision) in 7 patients, and biopsied only in the remaining 5 patients. The median dose of irradiation to the primary site was 30 Gy. RESULTS: With a median follow-up of 67 months (range 16-189 months) for the surviving patients, 28% failed distantly, and an additional 28% suffered from the progression of previously established metastatic disease. No patient failed locally. The median overall survival was 43 months. The actuarial overall survival at 5 years was 39% (+/-11%) for all patients and 60% (+/-14%) for patients with localized disease. CONCLUSIONS: Limited surgery and postoperative irradiation are one strategy that promises to balance the goal of achieving local control with the goal of diminishing late effects. Apart from the scenario in which radiation therapy is absolutely unnecessary, low-dose irradiation may be appropriate after considering the risk for local recurrence and overall prognosis.

Adolescent↗