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PubMed1999

Translocation of chromosomes 11 and 22 in choroidal metastatic Ewing sarcoma detected by fluorescent in situ hybridization.

PURPOSE: To describe a patient with metastasis of Ewing sarcoma to the choroid and the molecular genetics of the tumor. METHODS: A 26-year-old woman with metastatic Ewing sarcoma developed large choroidal masses in the left eye and died 2 months later. Autopsy of the eyes was performed. Dual-color fluorescent in situ hybridization was used to detect genetic alteration in the ocular tumor with EWS and FLI-1 probes. RESULTS: Histopathology confirmed choroidal metastatic Ewing sarcoma. Molecular analysis showed chromosomal translocation t(11;22)(q24;q12) or EWS/FLI-1 rearrangement in the malignant cells of the eye. CONCLUSIONS: Ewing sarcoma can rarely metastasize to the uvea. Molecular detection of the t(11;22)(q24;q12) translocation in Ewing sarcoma is valuable in the differential diagnosis of small round cell tumors.

Adult↗
PubMed2005

Tumor cell plasticity in Ewing sarcoma, an alternative circulatory system stimulated by hypoxia.

A striking feature of Ewing sarcoma is the presence of blood lakes lined by tumor cells. The significance of these structures, if any, is unknown. Here, we report that the extent of blood lakes correlates with poor clinical outcomes, whereas variables of angiogenesis do not. We also show that Ewing sarcoma cells form vessel-like tubes in vitro and express genes associated with vasculogenic mimicry. In tumor models, we show that there is blood flow through the blood lakes, suggesting that these structures in Ewing sarcoma contribute to the circulation. Furthermore, we present evidence that reduced oxygen tension may be instrumental in tube formation by plastic tumor cells. The abundant presence of these vasculogenic structures, in contrast to other tumor types, makes Ewing sarcoma the ideal model system to study these phenomena. The results suggest that optimal tumor treatment may require targeting of these structures in combination with prevention of angiogenesis.

Adolescent↗
PubMed2005

Intracranial Ewing sarcoma.

The occurrence of primary extraosseous Ewing sarcoma (EES) of the central nervous system (CNS) has only rarely been reported in the literature. It is important to distinguish this entity from the more common central primitive neuroectodermal tumor (PNET) of brain, since the management of these tumors is different from that of EES. We present the clinical, radiologic, and pathologic features of two cases of EES occurring in the brain. The diagnosis was further confirmed by detection of a rearrangement of the FLI1 and/or EWS gene loci in tumors from both patients using fluorescent in situ hybridization (FISH). Although rare, the possibility of EES should be considered particularly when tumors that arise near the meningeal surface of the brain and have the pathologic appearance of a PNET. Demonstration of t(11;22)(q24;q12) by molecular analysis essentially confirms the diagnosis and enables the oncologist to choose appropriate therapy.

Brain Neoplasms↗
PubMed1999

Tyrosine kinase Pyk2 mediates G-protein-coupled receptor regulation of the Ewing sarcoma RNA-binding protein EWS.

Ewing family tumors result from the effects of chromosomal translocations that fuse the Ewing sarcoma (EWS) gene to various genes encoding transcription factors. The resulting chimeric EWS fusion proteins are transcriptional activators with transforming potential that have received much study. By contrast, the cellular function of somatic EWS remains obscure. EWS belongs to a family of RNA-binding proteins thought to play role in RNA synthesis or processing. Here, we show that EWS interacts with Pyk2, a protein tyrosine kinase implicated in a variety of signal transduction processes. G-protein-coupled receptor signaling and other stimuli of Pyk2 kinase activity significantly block the interaction between EWS and Pyk2. Furthermore, as assessed by sucrose gradient centrifugation, EWS partitions with dense ribosome-containing fractions in a manner that is enhanced by signaling from the G-protein-coupled m1 muscarinic acetylcholine receptor (mAChR). We conclude that extranuclear EWS is a previously unrecognized target of G-protein-coupled receptor regulation.

Animals↗
PubMedDate not supplied

Treatment results and prognostic factors in Ewing sarcoma.

Files of 133 children with Ewing sarcoma (median age 10 years) were reviewed. Frequent primary sites were extremities, trunk, pelvis, and cranium. Half of 43 patients with metastases had disease in the lungs. Ten-year overall and event-free survival rates were 31% and 19%, respectively. Five-year overall survival rates were 42% in localized and 15% in metastatic disease (p < .0001); 66% in cases with primary tumors < 8 cm and 29% in larger tumors (p = .013). VAC (vincristine, actinomycin D, and cyclophosphamide) regimens with anthracyclines resulted in better survival. Presence of distant metastases, large primary tumors, and pelvic localization were related to poor prognosis. Novel therapeutic approaches are needed to produce better results, especially in high-risk patients.

Adolescent↗
PubMedDate not supplied

Ewing sarcoma family of tumors in unusual sites: confirmation by rt-PCR.

Ewing sarcoma family tumors originating in the palate or adrenal gland are extremely rare and may cause difficulty in diagnosis. More common tumors primary to these sites need to be excluded before one arrives at the correct diagnosis. We have recently diagnosed 2 such cases. The 1st case was that of a 24-year-old woman who presented with a swelling in the right side of the hard palate. The 2nd case was diagnosed in a 28-year-old woman who presented with a mass in the right adrenal gland. In both cases, the diagnosis of Ewing sarcoma family of tumors was confirmed by immunohistochemical studies and reverse transcriptase-polymerase chain reaction (RT-PCR). The hard palate case is the 1st and the adrenal gland the 3rd case of Ewing sarcoma family of tumors arising in these sites, in which the diagnosis was confirmed by RT-PCR and/or cytogenetics. Accurate diagnosis of Ewing sarcoma family of tumors is crucial for the management of patients, and when found in such rare locations, diagnosis should be supported by immunohistochemical and/or molecular genetic studies.

Adrenal Gland Neoplasms↗
PubMed2003

Outcome after relapse in an unselected cohort of children and adolescents with Ewing sarcoma.

BACKGROUND: Survival after relapse in patients with Ewing sarcoma is very poor and this retrospective study attempts to identify of prognostic factors predicting survival after relapse. PROCEDURE: A total of 191 patients with localised Ewing sarcoma were registered in the ET-2 trial of the United Kingdom Children's Cancer Study Group (UKCCSG). All patients received standardised primary treatment with chemotherapy and surgery and or radiotherapy as local modality treatment. Sixty-four patients who relapsed are included in this report. Treatment at relapse was variable and included chemotherapy, surgery, radiotherapy and high dose therapy (HDT) or megatherapy with peripheral stem cell transplantation (PBSCT) or autologous bone marrow transplantation (ABMT) in various combinations. A subgroup of patients had only non-specific symptomatic treatment at relapse. Both univariate and multivariate methods were used to investigate variables affecting survival after relapse. RESULTS: The overall actuarial median survival from relapse for all patients was 14 months (95% CI 11-16 months). Univariate analysis showed that males had a longer survival (median, 16 months vs. 11 months); patients who relapsed while on treatment did worse (median, 3 months vs. 16 months) and patients who had a longer disease-free interval (DFI) prior to relapse had a better outcome (DFI <1 year, median survival = 3 months; DFI 1-2 years, survival = 8 months; DFI > 2 years, median survival = 24 months, P < 0.001). Multivariate analysis confirmed that duration of first remission was the only factor associated with longer survival after relapse. CONCLUSIONS: These data suggest that although aggressive therapy may delay disease progression after relapse for some children, the course of the disease after relapse is usually fatal. International co-operative studies are needed to evaluate new strategies.

Adolescent↗
PubMed1994

Increased confidence of diagnosis of Ewing sarcoma using T2-weighted MR images.

To assess whether magnetic resonance imaging could assist in diagnosing Ewing sarcoma, we retrospectively analyzed T1- and T2-weighted images of 24 consecutive solitary lytic bone lesions, including 18 cases of histologically proven Ewing sarcoma, and six aggressive-appearing benign lesions including eosinophilic granuloma and osteomyelitis (3 cases each). Images were obtained using a (1.0T) Siemens GBSII system. No signal intensity differences were noted among the various lesions on T1-weighted images. The observed differences in T2-weighted marrow signal intensities were scored on a 5-point scale (5 = water, 3 = fat, and 1 = muscle) and then histologically correlated. Sixteen of 18 Ewing sarcomas showed homogeneous T2-weighted signal isointense with fat and two were heterogeneous. Signals for all 6 benign lesions were homogeneous and more intense than fat on T2-weighted images. All Ewing sarcomas had associated soft tissue masses whereas, only one benign lesion, an eosinophilic granuloma had a small soft tissue component. In our patient population, a homogeneous intramedullary T2-weighted signal isointense with fat, and the presence of an associated soft tissue mass helped differentiate Ewing sarcoma from benign lytic lesions. The reproducibility of these results requires testing in larger series of patients using a variety of MR imaging equipment of differing field strengths.

Adolescent↗
PubMed1997

Ewing sarcoma metastatic to the iris.

PURPOSE: To report a case of Ewing sarcoma metastatic to the iris. METHODS: A 19-year-old woman with metastatic Ewing sarcoma of the femur developed a diffuse, fluffy iris mass with a pseudohypopyon in the left eye. A fine-needle aspiration biopsy confirmed iris metastasis, and external beam radiotherapy was given to the affected eye. RESULTS: The iris mass responded initially to external beam radiotherapy and ongoing chemotherapy with vincristine, cyclophosphamide, doxorubicin, etoposide, and ifosfamide but recurred 5 months later. Subsequent radiotherapy with an iodine 125 plaque achieved further resolution of the iris tumor. CONCLUSIONS: Ewing sarcoma can rarely metastasize to the iris. The diagnosis can be confirmed by fine-needle aspiration biopsy. Plaque radiotherapy is a therapeutic alternative.

Adult↗
PubMedDate not supplied

[Ewing sarcoma--current opinion].

Ewing tumor family consists of Ewing tumor of bone, extraosseous Ewing tumor, primitive neurectodermal tumor and Askin tumor. All of them share genetic abnormality, reciprocal translocation (11; 22) (q24; q12), and originate from the same primordial stem cell. Ewing tumor is the most common form, found in 60% of cases. It is the second primary malignant bone tumor. Localized lesion is found in nearly 80% and metastatic disease in 20% of cases. Patients present mostly due to pain and palpable tumefaction, and pathological fracture as the initial problem develops in long bones. Ewing tumor can develop in virtually any bone of the body and in extraosseous localizations as well, while localization in the extremities occurs in 50% of patients. Head or neck localizations are extremely rare. Paraspinal, retroperitoneal or deep pelvic tumor localization is manifested by back pain. Systemic symptoms are also present, commonly fever or weight loss, which often indicates the presence of metastatic disease with predominant invasion of lung, bone and bone marrow. Multimodal chemotherapy with local radiation and/or surgical resection is the best way of modern treatment. Distal parts of extremities and axial skeleton are good prognostic features, while proximal parts, pelvic girdle, metastatic disease and low index of postchemotherapeutic necrosis are associated with poor outcome.

Bone Neoplasms↗
PubMed1984

Roentgenographic-pathologic correlation of diffuse sclerosis in Ewing sarcoma of bone.

Roentgenographic review of the first 210 cases of Ewing sarcoma (ES) in the Intergroup Ewing Sarcoma Study revealed that 37.6% of cases had evidence of diffusely increased intraosseous density or diffuse sclerosis (DS). In these cases the sclerosis was usually mixed with various patterns of lysis and/or combined with a periosteal reaction. A radiograph blinded histologic review of selected biopsies showed an 83% incidence of dead bone compared to 23% in those without DS. Ten percent of the cases with DS had appositional new bone formation on dead bone whereas none of the cases without DS showed such reactions. Pathologic explanation of the roentgenographically identified diffuse sclerosis in ES has not been previously well documented in the medical literature.

Bone Neoplasms↗
PubMed1986

Phenotypic characterization of Ewing sarcoma cell lines with monoclonal antibodies.

The histogenesis of Ewing sarcoma, the second most frequent bone tumor in humans, remains controversial. Four Ewing cell lines were analyzed by immunological methods. A panel of antibodies directed to T, B, and myelomonocytic markers gave negative results. Surface antigens recognized on Ewing cells were found to be related to the neuroectoderm lineage. Ganglioside GD2, a marker of neuroectodermal tissues and tumors, was present on all lines. These were also stained by the mouse monoclonal antibody HNK-1, which detects a carbohydrate epitope present on several glycoconjugates of the nervous system, including two glycoproteins, the myelin-associated glycoprotein and the neural cell-adhesion molecule (N-CAM), and an acidic glycolipid of the peripheral nervous system. The P61 monoclonal antibody, which reacts with a peptide moiety of N-CAM, and a rabbit antiserum, raised to purified mouse N-CAM and not recognizing the HNK-1-defined epitope, were also reactive. By contrast, all antibodies specific for hematopoietic cell surface antigens were totally negative. Besides these antigenic features, Ewing sarcoma cells are characterized by a specific t(11;22)(q24;q12) translocation also observed in neuroepithelioma, a neuroectodermal tumor, suggesting a possible evolutionary related origin. The recent finding that the human N-CAM gene is located at the vicinity of the breakpoint on chromosome 11 indicates that it might be involved in genetic rearrangements occurring in this region.

Antibodies, Monoclonal↗
PubMed1998

The histological response to chemotherapy as a predictor of the oncological outcome of operative treatment of Ewing sarcoma.

Seventy-four patients who had a Ewing sarcoma of bone were managed with preoperative and postoperative chemotherapy and operative resection, with or without postoperative irradiation. The primary objectives of the study were to determine the histological response to preoperative chemotherapy in terms of the percentage of tumor necrosis and to assess the relationship between the histological response and the oncological outcome. The minimum duration of follow-up of the surviving patients who were continuously free of disease was five years. Sections of each operative specimen were examined, and the histological response to chemotherapy was graded semiquantitatively. Grade I indicated necrosis of 50 per cent of the tumor or less; grade II, necrosis of more than 50 per cent but less than 90 per cent; grade III, necrosis of 90 to 99 per cent; and grade IV, necrosis of 100 per cent of the tumor. Of the seventy-four tumors, forty-four (59 per cent) were exquisitely sensitive to chemotherapy and had complete (grade-IV) or nearly complete (grade-III) necrosis. In contrast, fourteen tumors (19 per cent) had little or no response to chemotherapy (grade I) and sixteen (22 per cent) had a moderate degree of necrosis (grade II). The histological response to preoperative chemotherapy (p = 0.0001), followed by the size of the tumor (p = 0.001), were the most important predictors of event-free survival. At five years, the rate of event-free survival was zero of fourteen patients who had had a grade-I response, six of sixteen who had had a grade-II response, and thirty-seven (84 per cent) of forty-four who had had a grade-III or IV response. The risk of local recurrence was most strongly associated with the operative margins; there were only four local recurrences (6 per cent) after sixty-seven resections with negative margins. Local recurrence may also have been influenced by the histological response and the use of local radiation. There were no local recurrences after operative treatment of six tumors that had been associated with pathological fracture. The histological response to preoperative chemotherapy and the size of the primary tumor are the most important clinical predictors of the outcome of operative treatment of non-metastatic Ewing sarcoma. These indicators should be used to identify patients who are at high risk for metastasis as such patients may be candidates for more intensive or novel therapies.

Adolescent↗
PubMedDate not supplied

Spinal epidural extraskeletal Ewing sarcoma in an adolescent boy: a case report.

Extraskeletal Ewing sarcoma (EES) represents a rare soft tissue malignant neoplasm histologically similar to skeletal Ewing sarcoma. It occurs mainly in adolescents and young adults and commonly affects the paravertebral regions. The differential diagnosis includes other small, blue round cells tumors. The authors report a case of an EES involving the spinal epidural and paravertebral spaces in an adolescent boy. EES diagnosis was confirmed by features of histologic analysis and immunohistochemistry and by the presence of the t(11;22)(q24;q12) chromosomal translocation by reverse transcriptase-polymerase chain reaction.

Adolescent↗
PubMed2003

Complications in long-term survivors of Ewing sarcoma.

BACKGROUND: Multimodality treatment has dramatically improved the outcome of patients with Ewing sarcoma. However, there appears to be little information concerning treatment-related complications in patients who are long-term survivors. METHODS: Forty-one patients with Ewing sarcoma who were treated between 1960-1980 and who survived the disease by at least 20 years were included in the current study. In a retrospective analysis, all complications related to the multimodality treatment of Ewing sarcoma were assessed. RESULTS: The patient group was comprised of 17 men and 24 women, with a mean age at the time of presentation of 16.8 years (range, 5-51 years). Approximately 20% of the lesions were located in the pelvis. All but 9 patients (78%) received chemotherapy as part of their treatment. The overall follow-up period averaged 25 years (range, 20-36 years). All except 1 patient were alive at the time of final follow-up, with the latter patient dying of radiation-induced secondary malignancy after 33 years. Only 17 patients (41%) were found to be free of any complication. These included metastases, local recurrence, secondary malignancies, pathologic fractures, and radiation-associated and chemotherapy-associated morbidities. CONCLUSIONS: Although the patients in the current study were treated successfully in terms of surviving an aggressive tumor, the high complication rate in this group of long-term survivors is noteworthy and indicates that long-term follow-up should be mandatory.

Adolescent↗
PubMedDate not supplied

Epiphyseal primary location for osteosarcoma and Ewing sarcoma in patients with open physis.

Conventional osteosarcoma and Ewing sarcoma are the most common childhood malignant tumors of bone. These tumors arise in the metaphysis and diaphysis of long bones and may extend secondarily into the epiphysis. Primary epiphyseal location for these tumors is rare and may constitute a diagnostic and therapeutic challenge. The authors report two skeletally immature patients with an osteosarcoma and Ewing sarcoma limited to the epiphysis. Clinical, radiographic and histopathologic findings are discussed.

Adolescent↗
PubMed1999

Cytogenetic aberrations in Ewing sarcoma: are secondary changes associated with clinical outcome?

BACKGROUND: Ewing sarcoma is associated with a nonrandom pattern of primary and secondary chromosomal aberrations. Whereas the finding of rearrangements of chromosome 22, usually in the form of a balanced translocation t(11;22)(q24;q12), is important diagnostically, nothing is known about the potential prognostic impact of the secondary chromosomal aberrations. PROCEDURE: During a 1 3-year-period, short-term cultured tumor samples from 21 children and young adults with Ewing sarcoma were cytogenetically analyzed successfully. RESULTS: Clonal chromosome aberrations were detected in 18 patients, 17 of whom had the characteristic t(11;22)(q24;q12) or variants thereof. The most frequent secondary change was +8, followed by +12, +2, +5, +9, +15, and gain of material from the long and short arms of chromosome 1. The only recurrent secondary change that was restricted to tumors from the ten patients that were dead at latest follow-up was gain of 1q material. Furthermore, all three patients with tumors with chromosome numbers over 50 had died, and the only patient with a tumor karyotype lacking chromosome 22 rearrangement was alive without evidence of disease. CONCLUSIONS: These data and previously published results indicate that the karyotypic pattern not only may be of diagnostic significance but also may be important prognostically.

Adolescent↗