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PubMed1979

Ewing sarcoma: phalangeal primary with fatal cardiac metastases.

A 39-year-old man had pain and swelling of the terminal phalanx of a finger. Radiograph was interpreted as osteomyelitis, and amputation through the mid-phalanx was performed. Histology revealed Ewing sarcoma. Lung metastases rapidly developed. Right lung irradiation and systemic chemotherapy, including doxorubicin, were instituted. He developed progressive severe right ventricular failure which was attributed to effects of large pulmonary metastases. Autopsy showed massive right ventricular metastases, the primary pathological cause of the heart failure, without evidence of doxorubicin cardiomyopathy.

Adult↗

PubMed2006

STAT3 is activated in a subset of the Ewing sarcoma family of tumours.

STAT3 is an oncogene that regulates critical cellular processes and whose constitutive activation has been demonstrated to correlate with biological and clinical features in many types of human malignancy. In this study, STAT3 activation was assessed in the Ewing sarcoma family of tumours (ESFT), which is characterized by fusion of the EWS gene with one of several Ets transcription factors, most commonly EWS-FLI1. STAT3 activation was assessed by immunohistochemistry using a monoclonal antibody specific for tyrosine(705)-phosphorylated STAT3 (pSTAT3(tyr705)) and a tissue microarray containing 49 paraffin-embedded ESFT tumours with known EWS translocations. Twenty-five (51%) tumours were pSTAT3(tyr705)-positive, as defined by more than 10% tumour cell immunostaining. STAT3 activation correlated with tumour site at presentation, with pSTAT3(tyr705)-negative ESFT involving axial sites predominantly (p = 0.008). Notably, among 31 patients who presented with localized disease, high-level STAT3 activation correlated with better overall survival (p = 0.02). STAT3 activation was not directly related to EWS-FLI1 expression, since EWS-FLI1 transfection did not result in STAT3 activation. Furthermore, detailed molecular analysis indicated that STAT3 activation may be seen with EWS-FLI1 or EWS-ERG and appears to be independent of EWS-FLI1 fusion type. In conclusion, STAT3 activation is present in approximately half of ESFT and correlates with clinical features. The role of STAT3 activation in ESFT pathogenesis seems to be independent of the type of EWS/Ets translocation.

Adolescent↗

PubMed1996

Mediastinal mass following chemotherapy in patients with Ewing sarcoma and osteosarcoma.

Thymic hyperplasia following combination chemotherapy for malignant disease is very uncommon in adolescents and adults. Our experience includes a thymic enlargement noted on the sequential computed tomography (CT) in three patients who were disease-free after chemotherapy for Ewing sarcoma (2) and osteosarcoma (1). The development of an anterior mediastinal mass after successful chemotherapy does not always imply relapse of malignant disease. To prevent inappropriate treatment, the possibility of benign aetiology must be considered.

Adolescent↗

PubMed2003

Expression and subcellular localization of Ewing sarcoma (EWS) protein is affected by the methylation process.

Ewing sarcoma (EWS) protein contains an N-terminal transcriptional activation domain (EAD) and a C-terminal RNA-binding domain (RBD). Recently, we had shown that EWS protein is not only localized in the nucleus and cytosol, but also on the surface of T cells and that its RBD is extensively asymmetrically dimethylated on arginine residues. Here we show that stimulation of T cells with phytohemagglutinin (PHA) caused a time-dependent 10-fold increase in expression of methylated EWS protein on the cell surface and a sixfold increase in the nuclei of peripheral blood mononuclear cells (PBMC). Mitogenic stimulation of malignant T cell lines, however, did not increase their inherently high expression of EWS protein. This expression seemed to correlate with methionine adenosyltransferase activity and S-adenosyl-L-methionine (AdoMet) utilization in PBMC and tumor cells and thus indicates dependence on the methylation process. Inhibition of methylation in normal and malignant cells with the methylation inhibitor adenosine dialdehyde (AdOx) resulted in a three to fivefold decreased expression of EWS protein not only in the nucleus but also on the cell surface. The inhibitory effect of AdOx was compensated and negligible in PBMC, but not in tumor cells if they were treated simultaneously with mitogenic PHA concentrations. The present findings indicate that expression of EWS protein in the various subcellular compartments is affected by the methylation process, in particular by the availability of intracellular AdoMet.

Adenosine↗

PubMed2007

Temozolomide and intravenous irinotecan for treatment of advanced Ewing sarcoma.

BACKGROUND: Preclinical models show sequence-dependent synergy with the combination of temozolomide and irinotecan, and a Phase I trial has demonstrated the combination to be tolerable and active in children with relapsed solid tumors. Because responses were seen in patients with Ewing sarcoma (ES) on that trial, additional patients were treated with this combination following study completion. PROCEDURE: We reviewed data from all ES patients treated with temozolomide and irinotecan at four institutions, including seven patients treated on the above Phase I trial. RESULTS: Sixteen patients received a total of 95 courses, with a median of five courses per patient. All patients had either progressive disease (PD) during initial therapy (n = 5) or relapse within 2 years of diagnosis (n = 11). Twelve patients had metastatic disease at diagnosis, including 5 with bone and/or marrow metastases. Patients received oral temozolomide 100 mg/m(2)/day on days 1-5 plus intravenous irinotecan 10-20 mg/m(2)/day on days 1-5 and 8-12, with courses repeated every 21-28 days. We observed 1 complete, 3 partial, and 3 minor responses in 14 evaluable patients, with a median duration of response of 30 weeks. Planned 21-day courses were tolerable and no more toxic than 28-day courses. Myelosuppression was minimal despite heavy pretreatment. Grade 3-4 diarrhea occurred in 11% of courses and was related to higher irinotecan doses. Over 600 irinotecan doses were administered uneventfully at home. CONCLUSIONS: Temozolomide and protracted intravenous irinotecan given in 21-day courses was tolerable and active in patients with advanced ES. Home administration of irinotecan with temozolomide was safe and is reasonable palliative therapy. A formal Phase II study using a uniform dose and schedule is warranted to better define activity.

Administration, Oral↗

PubMed1986

[Diagnosis of Ewing sarcoma of the thoracic spine--differential diagnostic difficulties].

Spinal Ewing's sarcomas are rare and cause problems in differential diagnosis. The radiologic, nuclear medicine and CT findings in two children with histologically proven Ewing's sarcoma are presented and problems in differential diagnosis discussed. Biopsy should be done early.

Adolescent↗

PubMed2007

Adjuvant and neoadjuvant chemotherapy for Ewing sarcoma family tumors in patients aged between 40 and 60: report of 35 cases and comparison of results with 586 younger patients treated with the same protocols in the same years.

BACKGROUND: The clinical and pathologic features of 46 patients 40 to 60 years old with Ewing sarcoma family tumor (ESFT) diagnosed at the authors' institute between 1972 and 2000 were reviewed. METHODS: Ten patients with metastatic tumors at presentation went elsewhere for treatment; 35 of 36 remaining cases with localized disease were treated at the authors' institution according to different chemotherapy protocols activated in successive years. In patients with nonmetastatic tumors local treatment was surgery in 9 patients, radiotherapy in 16, and surgery followed by radiotherapy in 10. RESULTS: At follow-up times ranging from 6 and 34 years (mean, 17.8 years), 15 patients (42.9%) remained continuously disease-free, 19 experienced recurrence, and 1 died of chemotherapy-related toxicity. The 5- and 10-year event-free survivals were 42.9% and 35.2%, respectively, and the 5- and 10-year overall survivals were 46.1% and 42.8%, respectively. Comparing this group of patients with 586 cases of younger patients seen in the same period at Rizzoli, the only difference between the 2 groups was a significantly higher rate of tumors located in the soft tissues with a larger volume in the older group. The results achieved were comparable in the 2 groups, although the older group had a lower chemotherapy dose-intensity and a higher rate of WHO grade 4 hematologic toxicity. CONCLUSIONS: For patients with localized disease treated with adjuvant and neoadjuvant chemotherapy the results were essentially comparable in the 2 groups. It is concluded that patients 40 years or older with ESFT should be treated in the same way as younger patients and included in treatment trials for these tumors.

Adult↗

PubMed2002

Fine needle aspiration cytology in the diagnosis and management of children and adolescents with Ewing sarcoma and peripheral primitive neuroectodermal tumor.

PURPOSE: The utility of fine needle aspiration cytology (FNAC) in conjunction with immunocytochemistry in the diagnosis of Ewing sarcoma and peripheral primitive neuroectodermal tumor, the Ewing family of tumors (EFT), is retrospectively described. PATIENTS AND METHODS: During a 10-year period 24 children and adolescents were diagnosed at Karolinska Hospital to have EFT of bone or soft tissue using FNAC. Criteria for diagnosis was based on cytomorphology combined with immunocytochemistry. The median age was 14.1 years (range 0.7-20.2). FNAC was performed within a median time of 1 day after referral. RESULTS: Forty aspiration procedures were performed, 24 at primary work up in 23 patients and 16 at suspected relapses in 10 patients. A primary cytologic diagnosis of EFT was obtained in 22 of 23 cases. In nine cases with primary disease there was no histologic confirmation. Two tumors were on FNAC diagnosed as neuroblastoma versus EFT, and EFT, respectively. Histopathology on resected tumor tissue from these patients showed EFT and small cell osteosarcoma, respectively. Suspected relapse was found to be positive at five and negative at 11 occasions. Immunocytochemistry was positive for CD45 (LCA) in 0/12, for desmin in 2/21, for MIC2 in 15/15, for NB84 in 1/3, for NFP in 7/7, for NSE in 12/18, for S-100 in 4/11 and for vimentin in 18/19. CONCLUSIONS: The results show that FNAC together with immunocytochemistry is a rapid, physically atraumatic and accurate method in diagnosing both primary EFT of bone and soft tissue as well as relapses.

Adolescent↗

PubMed2006

Acquired von Willebrand syndrome in a patient with Ewing sarcoma.

Acquired von Willebrand syndrome is a rare bleeding disorder with clinical and laboratory features closely resembling to hereditary von Willebrand disease. The syndrome may accompany various conditions, including malignant disorders, most often with Wilms tumor and adrenal cell carcinoma. In this report, the authors present a patient with AvWS in association with Ewing sarcoma for the first time in the literature. The abnormal bleeding tendency was successfully treated with fresh frozen plasma and did not recur after the first two courses of chemotherapy with clinical improvement.

Adolescent↗

PubMed2002

Vertebra plana caused by primary Ewing sarcoma: case report and review of the literature.

A 7-year-old boy presented with vertebra plana of T11. The presumptive diagnosis suggested by clinical presentation, conventional radiographs, and computed tomographic scans was eosinophilic granuloma. Progressive neurologic symptoms required surgical excision of the lesion and decompression. Histopathologic examination of the surgical specimen confirmed the diagnosis of Ewing sarcoma.

Back Pain↗

PubMed2006

Sensitive Ewing sarcoma and neuroblastoma cell lines have increased levels of BAD expression and decreased levels of BAR expression compared to resistant cell lines.

The purpose of the study is to compare the mRNA expression of pro-apoptotic and anti-apoptotic genes by using the GeneSystem 320 (Capital Genomix Inc., Gaithersburg, MD) to examine the differential expression in Ewing sarcoma and neuroblastoma cell lines. This is an alternate method for which internal controls have been built into the system for comparing mRNA. The tumor cell lines were chosen based on their previously characterized Fas-resistance or Fas-sensitive properties in order to determine the differences in their response to apoptotic signals. Two representative pro-apoptotic genes (BAD and SMAC) and one anti-apoptotic gene (BAR) were chosen for the study. The results of mRNA expression were correlated with protein expression by Western analysis. BAD was highly expressed in the Fas-sensitive cell lines while SMAC was equally expressed in both Fas-sensitive and Fas-resistant cell lines. On the other hand, BAR was highly expressed in Fas-resistant cell lines and minimally expressed in the Fas-sensitive cell lines. Our data suggests that levels of BAD and BAR mRNA expression predict sensitivity to apoptosis.

Adaptor Proteins, Signal Transducing↗

PubMedDate not supplied

Numb chin syndrome in Ewing sarcoma.

The numb chin syndrome consists of unilateral hypesthesia of the chin and lower lip. In adults, it is often associated with metastatic disease to the mandible, base of the skull, or leptomeninges. In children, it has been associated with infiltration of the inferior alveolar nerve by leukemic cells. We describe two cases of numb chin syndrome in children with Ewing sarcoma. In a child with a solid tumor, this symptom seems to have an ominous meaning and should lead to the investigation of progressive skeletal involvement.

Adolescent↗

PubMed1994

DNA-binding and transcriptional activation properties of the EWS-FLI-1 fusion protein resulting from the t(11;22) translocation in Ewing sarcoma.

The 5' half of the EWS gene has recently been described to be fused to the 3' regions of genes encoding the DNA-binding domain of several transcriptional regulators, including ATF1, FLI-1, and ERG, in several human tumors. The most frequent occurrence of this situation results from the t(11;22)(q24;q12) chromosome translocation specific for Ewing sarcoma (ES) and related tumors which joins EWS sequences to the 3' half of FLI-1, which encodes a member of the Ets family of transcriptional regulators. We show here that this chimeric gene encodes an EWS-FLI-1 nuclear protein which binds DNA with the same sequence specificity as the wild-type parental FLI-1 protein. We further show that EWS-FLI-1 is an efficient sequence-specific transcriptional activator of model promoters containing FLI-1 (Ets)-binding sites, a property which is strictly dependent on the presence of its EWS domain. Comparison of the properties of the N-terminal activation domain of FLI-1 to those of the EWS domain of the fusion protein indicates that EWS-FLI-1 has altered transcriptional activation properties compared with FLI-1. These results suggest that EWS-FLI-1 contributes to the transformed phenotype of ES tumor cells by inducing the deregulated and/or unscheduled activation of genes normally responsive to FLI-1 or to other close members of the Ets family. ES and related tumors are characterized by an elevated level of c-myc expression. We show that EWS-FLI-1 is a transactivator of the c-myc promoter, suggesting that upregulation of c-myc expression is under control of EWS-FLI-1.

Amino Acid Sequence↗

PubMed2004

Ewing sarcoma: the pediatrician's point of view.

BACKGROUND: Integrated multimodal care is needed for patients with Ewing sarcoma, which is the second most common primary bone malignancy in children and adolescence. Chemotherapy increases survival from less than 5% to 65-70% for patients with localized tumors and to 25-30% for those with metastases at diagnosis. Surgery is a major tool, whereas advances in imaging techniques have improved the indications for and the optimization of treatment. Radiotherapy remains useful, either alone or in addition to surgery, and new techniques (conformational RT and IMRT) will reduce short-term toxic effects. Pediatric oncologists do not outweigh surgeons or radiation therapists, but they are the ones who coordinate the medical team, which also includes pathologists and imaging specialists. METHODS: The point of view of the pediatric oncologist was assessed as follows: the place of chemotherapy in Ewing tumor treatment, the place of radiotherapy in Ewing tumor treatment (including why avoid radiotherapy when technically possible in children), and how to proceed into the future? The place of surgery as local treatment for Ewing tumors was also evaluated. RESULTS: These reviews show a dynamic and kaleidoscopic panorama of intense activity at the laboratory and clinical levels. CONCLUSIONS: Though good survival rates have been achieved, improvements using entirely new approaches are needed.

Algorithms↗

PubMed1979

Ewing sarcoma: treatment with high dose radiation and adjuvant chemotherapy.

Twenty-one patients with pathologically proven Ewing sarcoma without overt metastases at diagnosis were treated with a protocol designed by the Royal Marsden/St. Bartholomew's Hospitals Children's Solid Tumour Group (CSTG). They received megavoltage radiotherapy to the involved bone and adjuvant chemotherapy with a combination of four cytotoxic drugs. Seven patients have so far relapsed, four at the original site and three in other bones. The other 14 are clinically free of disease a median of 36 months from diagnosis. Comparison with a historical control group of 19 patients treated with surgery or radiotherapy, but without initial chemotherapy, shows a significant improvement in survival for the study group (P = 0.03). Seventeen of the controls have died. The treatment regime was moderately toxic, but there were no treatment-related deaths. These results confirm that an improved survival time and hopefully cure rate can be expected from treating Ewing tumour with high doses of megavoltage radiation and combination chemotherapy. Future goals must be the better control of large primary lesions and the eradication of micrometastases in other bones. The place of surgery should be re-evaluated in the treatment of the primary tumour, and better adjuvant chemotherapy regimes are needed.

Adolescent↗

PubMed2001

Intramedullary Ewing sarcoma of the spinal cord: consequences of molecular diagnostics. Case report.

Molecular biological techniques have begun to transform modern medicine. These techniques have shown promise in the pathological diagnosis of difficult or uncommon tumors. Accurate molecular diagnosis of the small round-cell tumors, for example, is especially important because divergent therapies may be required to eradicate such disparate lesions as neuroblastoma, lymphoma, rhabdomyosarcoma, central primitive neuroectodermal tumors/medulloblastoma, or Ewing sarcoma (ES). The authors present an unusual case of a primary, extraosseous ES arising from the intramedullary spinal cord, in which molecular studies were required for specific diagnosis and therapeutic guidance.

Adult↗

PubMed2006

Novel approaches for the management of patients with Ewing sarcoma.

The concepts of tailored therapy according to genetic profiling and response based on minimal residual disease evaluation during therapy are attracting increasing interest in modern clinical oncology. Children with acute lymphoblastic leukemia are being stratified to various treatment arms with different intensities according to the genetic characteristics of their leukemia and their response to therapy as measured by real-time polymerase chain reaction. Our ability to quickly identify patients with Ewing sarcoma who have a poor prognosis, and to offer them aggressive therapeutic modalities, such as stem cell transplantation, may result in an improved cure rate. Based on the knowledge gained by gene expression profiling and gene silencing techniques we can expect the emergence of new specific drugs that will target malignant cells without causing damage to normal tissue, resulting in improved cancer therapy.

Disease-Free Survival↗

PubMed1989

Human differentiation-stimulating factor (leukemia inhibitory factor, human interleukin DA) gene maps distal to the Ewing sarcoma breakpoint on 22q.

The human gene encoding differentiation-stimulating factor (D-factor) has previously been isolated and shown to be identical to leukemia inhibitory factor (LIF). We have determined a fine structure map of approximately 20-kb surrounding the D-factor/LIF gene. Southern blot analysis using a somatic cell hybrid panel shows that the gene maps to chromosome 22. D-factor/LIF was further sublocalized to 22q11.2----q13.1, distal to a Ewing sarcoma (ES) breakpoint, using a second somatic cell hybrid panel. Probes to the 5' and 3' regions of the locus and the cDNA were used to screen for restriction fragment length polymorphisms, but none were detected. Analysis by pulsed field gel electrophoresis suggests that D-factor/LIF is not near the ES breakpoint.

Animals↗