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PubMed1996

Treatment of femoral Ewing's sarcoma.

BACKGROUND: The treatment of Ewing's sarcoma consists of chemotherapy for systemic and local disease. However, the role of radiation therapy, and/or surgical resection for definitive local treatment has yet to be determined. METHODS: A retrospective review of 32 patients (24 males and 8 females) treated for femoral Ewing's sarcoma between 1970 and 1985 was performed. Patients were divided into 3 treatment groups: chemotherapy and radiotherapy (CR) (10); chemotherapy and surgery (CS) (9); and chemotherapy, surgery, and radiotherapy (CSR) (13). Patients in the CR group received a mean of 5320 centigray (cGy) of radiation and patients in the CSR group received a mean of 3590 cGy. Multiagent cyclophosphamide/doxorubicin based chemotherapy was used in all cases. Surgery consisted of wide resection or amputation. RESULTS: Patients in the CR group had a higher risk of local recurrence than patients in the CS and CSR groups (P=0.02, log rank). The combination of local recurrences and treatment complications necessitated surgery for 7 of 10 CR patients, whereas 1 of 9 and 4 of 13 in the CS and CSR groups required additional surgery. The median survival for the entire group was 39 months. Minimum follow-up for surviving patients was 45 months. Five-year survival consisted of 1 of 10 patients in the CR group, 2 of 9 in the CS group, and 7 of 13 in the CSR group. There were no statistically significant differences among the three survival curves. Tumor location within the femur was a significant prognostic variable. Distal femoral location had a survival advantage compared with proximal and mid-femur locations (P = 0.049, log rank). CONCLUSIONS: Femoral Ewing's sarcoma remains a disease with a poor prognosis. Radiation alone for local treatment results in a high rate of local recurrence and complications. Our current local treatment strategy for femoral Ewing's sarcoma includes surgery in all and adjuvant radiotherapy in many of the patients.

Adolescent↗
PubMed1995

Biphenotypic sarcomas with myogenic and neural differentiation express the Ewing's sarcoma EWS/FLI1 fusion gene.

Accurate diagnosis of primitive childhood sarcomas continues to be a formidable problem because these malignancies generally demonstrate very little morphological evidence of their tissue of origin. One of these tumor classes, the Ewing's sarcoma family of peripheral primitive neuroectodermal tumors (pPNETs), are thought to have a neural histogenesis based on evidence of neuroectodermal differentiation. Greater than 95% of pPNETs carry t(11;22) or t(21;22) chromosomal translocations which fuse the EWS gene from chromosome 22q12 in-frame with either FLI1 from chromosome 11q24 or ERG from chromosome 21q22. The pPNETs are considered to be histogenetically distinct from rhabdomyosarcomas, myogenic tumors lacking these EWS gene fusions and hypothesized to derive from immature skeletal muscle precursors. In the present study, we describe a unique set of childhood soft tissue sarcomas that show both neural and myogenic differentiation. These biphenotypic tumors express myogenic regulatory factors and muscle-specific antigens and also show neuroectodermal differentiation with ultrastructural evidence of neurosecretory granules and expression of neural-associated genes. Northern analysis and reverse transcriptase PCR reveal expression of EWS/FLI1 gene fusions in all biphenotypic sarcomas analyzed. Chimeric EWS/FLI1 transcripts and fusion proteins in these tumors are identical to those described for pPNETs. Our results provide evidence for a class of biphenotypic childhood sarcomas with myogenic and neural differentiation and suggest that these tumors may be related to the Ewing's sarcoma family of pPNETs.

Adolescent↗
PubMed1995

[The magnetic resonance tomography of Ewing's sarcomas: the morphology and tumor extension].

PURPOSE: The morphology and delineation of Ewing sarcoma in magnetic resonance imaging was investigated. METHODS AND MATERIALS: Magnetic resonance images (spin-echo techniques; T1-w pre/post gadolinium, T2-w) of 59 patients as part of a multicenter study were evaluated retrospectively. Qualitative image analysis was performed: signal intensity (point of reference extraosseous: muscle, intraosseous: bone marrow), enhancement patterns, lesion delineation and differentiation between tumor and oedema. RESULTS: Signal intensity: T1-w: extraosseous: 75% isointense, intraosseous: 92% hypointense; T2-w extraosseous: 100% hyperintense, intraosseous: 93% hyperintense. Enhancement pattern: 97% both extra- and intraosseous. Best delineation intraosseous in T1-w (53% good, 36% very good), extraosseous in gadolinium enhanced T1-w (46% good, 37% very good) and T2-w (55% good, 33% very good). Differentiation between tumour and oedema was intraosseous not possible, extraosseous in T2-w in 61%. CONCLUSION: Morphology of Ewing sarcoma in magnetic resonance imaging is rather uniform. The lesion is intra- and extraosseous sharply delineated, though tumour and oedema can be rarely differentiated.

Adolescent↗
PubMed1989

[Problems in the diagnosis of Ewing's sarcoma].

The aim of the work was to analyze by the method of retrospective study the difficulties in diagnosing the Ewing sarcoma at the Department of Orthopaedic of the Teaching Hospital in Bratislava in the years 1967-1987. The authors found out that there were diagnosticated 28 cases of the Ewing sarcoma, the boy-girl ratio was: 1:1. Most frequently it occurred between 12 and 18 years of age. Apart from clinical examination they use classical sciagraphy, macropictures, tomography, arteriography, gammagraphy of the skeleton, computer tomography, lymphography, histological, and electron microscopic examination. In the conclusion the authors point out that the timely treatment may be ensured by a precise and demanding screening carried out by pediatricians, practitioners in charge of adolescents and orthopaedists and by further reduction of the time interval necessary for performing all above mentioned examinations. The care of the Ewing sarcoma is the responsibility of suitably equipped special centres of bone oncology.

Adolescent↗
PubMed1989

Expression of a new human THY-1 related antigen in Ewing's sarcoma and peripheral neuroectodermal tumors.

Ewing's sarcoma (ES), peripheral neuroectodermal tumor (PNET) and Askin's tumor of the chest wall share a reciprocal chromosomal translocation between the long arms of chromosomes 11 and 22 (q23-24; q12). In the absence of other distinguishing features this specific translocation is regarded as marker of a common and neuroectodermal origin for these rare tumors. A monoclonal antibody (HBA-71) developed in our laboratory has been found to recognize an unique ES and PNET associated antigen, which is also expressed in some normal tissues, including thymus, bone marrow, islets of Langerhans, ependyma and adenohypophysis. It is shown in this study that this HBA-71 antigen is closely related to the murine THY-1 antigens, major cell surface glycoproteins of thymocytes and brain in mice and rat. Both antigens have similar molecular ratios (18,000), amino acid compositions and sensitivity to tryptic digestion, show high cell surface expression, and binding of the appropriate antibodies to HBA-71 antigen triggers proliferation in thymocytes. The HBA-71 epitope may represent a primitive neuroectodermal marker of ES/PNET, or its expression may be directly linked to the reciprocal translocation invariably associated with HBA-71-positive ES and PNET tumors, which maps to the same region of chromosome 11 (q23-24) as the human Thy-1 gene.

Amino Acids↗
PubMed1986

Primary spinal epidural Ewing's sarcoma.

A rare case of Ewing's sarcoma, originating primarily in the spinal epidural space is reported. Only five similar case reports are available in the English literature. Full recovery from a state of complete paraplegia, due to malignant compression, local recurrence without distant metastases and the longest survival of more than three years and six months, makes a unique combination. Pathology and therapy are briefly discussed and the pertinent literature reviewed.

Adolescent↗
PubMedDate not supplied

Extraosseous Ewing's sarcoma in the foot.

Extraosseous Ewing's sarcoma is an extremely rare neoplasm that may occur in the lower extremity. The neoplasm is highly aggressive and is usually fatal, however, early diagnosis and treatment greatly increase the chances of survival. A case report and review of the literature is presented.

Adult↗
PubMed2004

Prediction of high risk Ewing's sarcoma by gene expression profiling.

Ewing's sarcoma (ES) is the second most common primary malignant bone tumor in children and adolescents. Currently accepted clinical prognostic factors fail to classify ES patients' risk to relapse at diagnosis. We aimed to find a new strategy to distinguish between poor and good prognosis ES patients already at diagnosis. We analysed the gene expression profiles of 14 primary tumor specimens and six metastases from ES patients, using oligonucleotide microarray analysis. The over-expression of two genes was validated by quantitative PCR using the LightCycler system. We identified two distinct gene expression signatures distinguishing high-risk ES patients that are likely to progress from low-risk ES patients with a favorable prognosis of long-term progression-free survival. The microarray-based classification was superior to currently used prognostic parameters. Over-expressed genes in the poor prognosis patients included genes regulating the cell cycle and genes associated with invasion and metastasis, while among the downregulated genes were tumor suppressor genes and inducers of apoptosis. Our results indicate the existence of a specific gene expression signature of outcome in ES already at diagnosis, and provide a strategy to select patients who would benefit from risk-adapted improved therapy.

Adolescent↗
PubMedDate not supplied

[EICESS 92 (European Intergroup Cooperative Ewing's Sarcoma Study)-- preliminary results].

BACKGROUND: Ewing tumor patients' outcome is 50% to 60% with current treatment strategies. Questions concerning toxicity and secondary malignancies are of increasing importance. PATIENTS AND METHODS: 631 patients were registered with the German EICESS study center of the European Intergroup Cooperative Ewing's Sarcoma Study, 369 patients were randomized. Treatment apart from local therapy consisted of 14 courses of Vincristine, Actinomycin D, Cyclophosphamide or Ifosfamide, Adriamycin (Doxorubicin), with or without Etoposide. First results concerning event-free survival (EFS), toxicity, and the rate of secondary malignancies three years after diagnosis are presented. RESULTS: Three year EFS was 0.66 for patients with localized tumors, 0.43 for patients with primary pulmonary/pleural metastases, and 0.29 for patients with other metastases, respectively. Large tumor volume or pelvic site, especially if inoperable, were adverse prognostic factors. Both histological and MRT-defined response were positively correlated to outcome. Up to 67% of patients experienced WHO grade IV toxicity, mostly related to bone marrow depression. The treatment related mortality was 1% (6/631). Until 15.02.1999, six of 687 patients have suffered secondary malignancies, two of six after (additional) myeloablative therapy. CONCLUSIONS: EICESS 92 treatment is toxic, but manageable and compares favorably to international results. New strategies must be sought for certain risk groups of patients.

Adult↗
PubMed2000

Primary petrous bone Ewing's sarcoma.

A case of primary Ewing's sarcoma involving a large portion of the petrous temporal bone in an eighteen month old child is reported. The lesion was treated by radical surgery and adjuvant chemotherapy and radiotherapy. An extremely rare age of presentation, an unusual location and an excellent response to treatment are the highlights of this case.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2000

Orbital Ewing's sarcoma of the orbit.

PURPOSE: Ewing's tumor is a primary tumor of bone in childhood that only rarely involves the orbit. Most such cases are metastatic from distant sites. This tumor may be confused with other small round cell malignancies of childhood, and immunohistochemical studies are essential in making the diagnosis. METHODS: We present two cases of Ewing's tumor of the orbit. One was in a 22-year-old boy with an occult primary tumor in the frontal bone that became symptomatic after forehead trauma. The other example was in a 7-year-old boy with a known Ewing's primary of the clavicle. The clinical manifestations and diagnostic criteria are discussed. RESULTS: In most cases with orbital involvement, ophthalmic symptoms consist of proptosis, pain, and occasionally visual loss and motility restriction. The diagnosis is typically unsuspected before histologic evaluation. Electron microscopic and immunohistochemical analyses are essential in making the diagnosis and are necessary for all such small round cell tumors. CONCLUSIONS: Ewing's sarcoma is a rare orbital tumor of bone mainly affecting children. Local treatment relying on surgical extirpation and radiotherapy alone has proven inadequate, with 5-year survival rates of <10%. The addition of chemotherapy has improved survival rates significantly to approximately 50%.

Biopsy↗
PubMed1989

Chromosome 22 abnormalities in Ewing's sarcoma.

A child with disseminated Ewing's sarcoma underwent cytogenetic investigations which showed different structural rearrangements of chromosome 22 at diagnosis (?ring22), and at relapse [t(10;22)], but the classic translocation t(11;22) was not detectable. This case provides further evidence of the importance of chromosome 22 in this disease, while raising some questions about the central importance of the translocation between chromosomes 11 and 22.

Bone Neoplasms↗
PubMed2002

Detection of chimeric genes in Ewing's sarcoma and its clinical applications.

Ewing's sarcoma (ES), most commonly an undifferentiated tumor of bone, belongs to the enigmatic diagnostic category of small round cell tumors (SRCT) of childhood. The consistent presence of the translocation t (11; 22) in the vast majority of tumors provides evidence for a common histogenesis in ES and its family of tumors (ESFT), and also provides a unique diagnostic characteristic to discriminate this tumor family from SRCT. Molecular analysis of this translocation has revealed that it forms a chimeric gene between EWS on chromosome 22 and FLI-1 on chromosome 11. Similarly, the variant t (21; 22), t (7; 22), t (17; 22), and t (2; 22) rearrangements also form chimeric genes between regions of EWS and the ETS gene family (ERG, ETV1, E1AF, and FEV). Detection of these specific chimeric genes would provide a method for diagnosis of ESFT. We have developed a procedure for simultaneous detection of the chimeric genes by reverse transcription polymerase chain reaction (RT-PCR) with a mixture of primers. We conclude that the detecting those chimeric genes by this method can be easy and useful for diagnosis of ESFT. Moreover, by defining the specific chimeric gene it is possible to detect the tumor cell contamination in autologous blood stem cell transplantation.

Bone Neoplasms↗
PubMed1998

[Rhabdomyosarcoma and extraosseous Ewing's sarcoma].

Rhabdomyosarcomas (RMS) and extraosseous Ewing's sarcomas (EOE) including malignant peripheral neuroectodermal tumors (MPNT) are the most frequent soft tissue malignancies of childhood. They account for 60.2% of 2.350 cases collected in the files of the Kiel Pediatric Tumor Registry. RMS: It is absolutely necessary to distinguish between embryonal (e) and alveolar (a) RMS, since these are two distinct tumor entities with significant differences in clinical presentation, morphology, molecular biology, cytogenetics and prognosis (Botryoid and spindle cell RMS are special variants of eRMS). The overall proportion of eRMS: aRMS is 2.4:1. Most cases of eRMS develop in the first 10 years of life (77.3%) while the age distribution of aRMS is almost constant in childhood and adolescence. Embryonal RMS exhibit a significantly higher proportion of male patients than aRMS (m:f = 1.72:1 vs. 1.06:1). A higher percentage of aRMS cases (25%) shows metastatic disease at the time of diagnosis than eRMS (8%), and the overall survival rates of aRMS are significantly lower even in localised disease (stage I-III) than in eRMS (59% vs. 76%; p < 0.002) (Data from the Cooperative Soft Tissue Sarcoma Study CWS). EOE: Despite proven histogenetic relationship (identical chromosomal rearrangements and fusion genes) the members of the Ewing's sarcoma (ES) family, classic ES and MPNT, whether osseous or extraosseous display significant differences in location, morphology and prognosis. Morphologically, MPNT and classic ES can be considered to be the extremes of a spectrum with overt neurodifferentiation in the former and lack of neural differentiation in the latter. Matched-pairs analysis of CWS EOE and MPNT cases show dramatically more unfavorable overall survival rates in MPNT than in EOE (45% vs. 67%).

Adolescent↗
PubMed1988

Characterization of a human endocrine tissue and tumor-associated Ewing's sarcoma antigen.

The histogenesis of Ewing's sarcoma (ES), the second most frequent primary bone tumor in humans, remains controversial. A new cell line (SIM-1) was derived from a peripheral neuroectodermal tumor (PNET) and used for the production of a monoclonal antibody (HBA-71), which recognizes a novel cell surface antigen of ES- and PNET-derived cells and paraffin-embedded tumor sections. The HBA-71 antigen expression is restricted to PNET/ES and the antigen was not detected on cell lines or tissue sections of any other tumor tested, with the exception of ependymoma. Three proteins with molecular weights of 300,000, 185,000, and 90,000 were isolated from SIM-1 membrane extracts by HBA-71 affinity chromatography. Trypsin treatment of intact SIM-1 cells destroys the HBA-71 epitope and cleaves off two proteins with molecular weights of 210,000 and 95,000. HBA-71 antigen expression is not influenced by treatment of ES cell lines with differentiation inducers. Within normal tissues reactivity was observed with the adenohypophysis, ependymal cells, endocrine pancreas, Sertoli, and ovary granulosa cells. The reagent links ES with PNET and provides a highly valuable probe for (a) the immunohistological differential diagnosis of ES/PNET using fresh tissue or paraffin sections from other small round cell tumors, (b) the histogenetic studies of ES/PNET, and (c) the in vivo diagnostic and therapeutic procedures in patients with ES and PNET.

Adolescent↗
PubMed1988

Immunohistological characterization of a Ewing's sarcoma case.

The histogenesis of Ewing's sarcoma (ES) remains uncertain. Mesenchymal and neuroectodermal origins were the most recent hypotheses. In an attempt to test these two hypotheses, frozen sections of an ES with the chromosomal translocation t(11;22) have been studied using a panel of antibodies directed against monocytes/macrophages cell surface antigens (Leu M1, Leu M2, Leu M3, and MO1), and against neural components (NSE, S-100, T4, and HNK-1). None of these antigens were detected. Positive reactions were obtained with antibodies recognizing HLA II antigen and B2-microglobulin. From a panel of various intermediate filaments only vimentin was shown to be present. None of the two hypotheses could be supported by the results obtained from the immunohistological analysis of the tumor studied. In the absence of a specific immunological pattern, the chromosomal t(11;22)(q24;q12) marker remains the only diagnostic criterion of ES.

Antibodies, Monoclonal↗