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PubMed2007

Early lymphocyte recovery as a prognostic indicator for high-risk Ewing sarcoma.

BACKGROUND: Increasing evidence suggests that lymphocyte recovery plays a major part in tumor control. Facilitating immune reconstitution might be a novel direction of cancer therapy. The purpose of this study was to determine if early lymphocyte recovery is an independent prognostic indicator for high-risk Ewing sarcoma outcome. RESULTS: Data of 24 Ewing sarcoma patients were analyzed (age, 3 to 50 y; median, 16.5; male to female, 16:8). The 5-year overall survival (OS) of the total population was 47.9% [10.6 standard error (SE)]. Patients were separated into 2 groups: prolonged lymphopenia versus early lymphocyte recovery, using a threshold absolute lymphocyte count (ALC) of > or =500 cells/microL on day 15. The majority (67%; n=16) of the patients had an ALC > or =500 cells/microL, and of these 10/16 are alive with a 5-year OS of 58.7% (13.2 SE). In contrast, 33% (n=8) of patients had an ALC <500 cells/microL on day 15 and only 2/8 are alive with a 5-year OS of 25% (15.3 SE). This difference was significant (P=0.007 using the log rank test). When comparing patients with metastatic disease, patients with an ALC-15 < 500 cells/microL had a median survival of 13 months, whereas patients with an ALC-15 > or =500 cells/microL had a median survival of 29.5 months. All patients had an ALC before chemotherapy of >1000 cells/microL. The difference was significant (P value=0.001 using the log rank test). Univariate analysis of platelet counts, age, sex, and absolute neutrophil count showed no statistically significant association with OS. CONCLUSIONS: The data demonstrate that an ALC > or =500 cells/microL on day 15 of the first course of chemotherapy is an independent prognostic factor associated with superior OS in high-risk Ewing sarcoma.

Adolescent↗

PubMed2002

Pilot trial of tumor-specific peptide vaccination and continuous infusion interleukin-2 in patients with recurrent Ewing sarcoma and alveolar rhabdomyosarcoma: an inter-institute NIH study.

BACKGROUND: Patients with recurrent Ewing sarcoma and alveolar rhabdomyosarcoma have poor prognoses and limited therapeutic options. We have investigated the use of peptide pulsed vaccination in an attempt to immunologically target the breakpoint region of tumor specific fusion proteins expressed in these tumors. PROCEDURE: Sixteen patients with recurrent, translocation positive, Ewing sarcoma, and alveolar rhabdomyosarcoma underwent apheresis for collection of peripheral blood mononuclear cells. Following countercurrent centrifugal elutriation, an apheresis product comprised predominantly of monocytes but containing small numbers of circulating immature dendritic cells was pulsed with peptides derived from the breakpoint region of the fusion proteins. Vaccines were administered intravenously concomitant with continuous intravenous rhIL-2 at 9 x 10(6) IU/m(2)/day. RESULTS: Toxicity was limited to IL-2 related effects and was generally mild. Following vaccination, all patients showed progressive disease, most in a rapid fashion following the first vaccine. One patient showed evidence of an immunologic response and another showed a mixed clinical response. Patients enrolled on this tumor vaccine trial showed significant immunosuppression and large bulky tumors. CONCLUSIONS: Peptide vaccination as administered in this trial did not alter the dismal clinical outcome for patients with recurrent pediatric sarcomas. Future trials of tumor vaccines in this population should target patient populations with improved immune competence and smaller tumor burdens. Furthermore, optimization of the antigen presenting cell populations may be important for inducing immune responses to peptide antigens.

Adolescent↗

PubMed1998

The genomic breakpoint and chimeric transcripts in the EWSR1-ETV4/E1AF gene fusion in Ewing sarcoma.

Chromosome translocation creates a fusion between the EWSR1 gene and an ETS family gene. The fusion between these two genes is a characteristic feature of Ewing sarcoma. We previously identified a fourth translocation, t(17;22)(q12;q12), in genomic DNA isolated from cells of patients affected with Ewing sarcoma. The discovery of this translocation suggested that there might be a novel EWSR1-ETV4 fusion gene. In the present study, we determined the genomic breakpoint and characterized the chimeric transcript of the EWSR1-ETV4 fusion gene in two t(17;22) Ewing sarcomas. Reverse transcriptase-PCR assay showed an in-frame fusion between the 5'-terminal region of EWSR1 and the 3' end of ETV4 (alias E1AF, PEA3); the chimeric transcript could thus serve as a template for expression of a protein composed of the N-terminal portion of EWSR1 fused to the DNA-binding domain of ETV4. Long PCR and sequence analysis of genomic DNA revealed that either exon 8 or intron 7 of EWSR1 is fused to the same intron of ETV4 in both tumors. Several palindromic oligomer sequences were found close to the breakpoints in both genes. The 159-bp Alu-like sequence was repeated in the breakpoint region of the ETV4 gene. These observations suggest a mechanism of EWSR1-ETV4 gene fusion.

Alu Elements↗

PubMed1981

Lumbar epidural Ewing sarcoma. Light and electron microscopic investigation.

The clinicopathological findings in a child with extraskeletal Ewing sarcoma are described. The patient complained of pain in the lower back and difficulty walking. An extraskeletal, epidural, friable tumor, 2-3 cm long was removed from the epidural space. It had no relationship with the bone structures. Light and electron microscopic examination of the tumor led to the diagnosis of Ewing sarcoma. The morphological aspects of this neoplasia and the problem of the differential diagnosis with other small cell tumors of the epidural space are discussed.

Adolescent↗

PubMed1997

Differences in dose scheduling as a factor in the etiology of anthracycline-induced cardiotoxicity in Ewing sarcoma patients.

Clinical observation suggested a high prevalence of cardiac morbidity and mortality in children with Ewing sarcoma (ES) treated at B.C.'s Children's Hospital. We therefore compared 30 patients treated for Ewing sarcoma between 1978 and 1991 with 26 soft tissue sarcoma (STS) patients treated with similar chemotherapy over the same period of time. All patients were evaluated for cardiac function using echocardiography. Shortening fraction (SF) and left ventricular mass index (Massl) were compared before and after treatment. The role of chest irradiation, dose concentration (DC) of adriamycin (AD), total mean doses of AD, cyclophosphamide (CY) and actinomycin (AC) were analysed. SF for patients with ES and STS postchemotherapy was significantly lower (P < .001 and P = 0.0004, respectively) than pretreatment values. Postchemotherapy SF for ES was lower than STS (P = 0.0097). Massl for each group did not change significantly. Six of the ES patients had postchemotherapy SF of < 0.20, with three in congestive failure, two cardiac deaths and one heart transplant. One additional ES patient had sick sinus syndrome and needed a pacemaker. Among the STS patients only one had SF < .20 and none were symptomatic. There were no significant differences in the mean AD, CY and AC doses for ES versus STS. The difference in the DC of AD for ES (mean 744) compared to STS (mean = 362) was significant (P = < 0.001). Regression analysis indicated a trend for decreasing SF with increasing DC (P = 0.017). Chest irradiation did not appear to increase the likelihood of cardiotoxicity. ES patients had a higher prevalence of cardiac dysfunction compared to STS. Studies are required to evaluate the importance of the components of DC, i.e., size of the individual dose and frequency of administration of AD, and to look at other possible factors in the causation of cardiomyopathy in ES.

Anthracyclines↗

PubMed2005

Second malignancy in 597 patients with ewing sarcoma of bone treated at a single institution with adjuvant and neoadjuvant chemotherapy between 1972 and 1999.

The relative risk of second tumors in patients with Ewing sarcoma is controversial, and little is known about their treatment and outcome. The purpose of the current study was to define the incidence and features of second tumors among 597 long-term survivors of nonmetastatic Ewing sarcoma treated with adjuvant and neoadjuvant chemotherapy, radiotherapy, and/or surgery. The authors found that the risk of secondary malignancy after adjuvant or neoadjuvant treatment of Ewing sarcoma is higher than that after other childhood or adolescent cancers only after radiotherapy. Based on this, postoperative radiotherapy should be avoided when surgery with adequate margins is feasible.

Adolescent↗

PubMed1980

Ewing sarcoma: its course and treatment in 50 adult patients.

34 patients were treated for Ewing sarcoma with primary presentation. There were 10 females and 24 males with average age of 21 years (range 16-36 years). These patients received radiotherapy (greater than 5,000 R) to the primary site and combination chemotherapy with vincristine, cyclo-hosphamide, doxorubicin and actinomycin D. Of 19 patients with primary presentation in an extremity, 12 remain alive at 29 months without clinical evidence of tumor (63%); 2 patients are alive with relapse. All 10 patients with primary presentation in the pelvis died despite vigorous treatment. 3 patients are alive out of 5 patients with primary presentation in chest or abdominal wall or axilla but only 1 patient remained tumor-free. Of the entire group of 34 patients, 12 remained tumor-free at 27 months (35%). 2 relapsed patients were rendered surgically tumor-free and receive further treatment. 20 patients died or are receiving treatment for advancing disease. 16 patients were treated for locally recurrent (1 patient) or metastatic (15 patients) Ewing sarcoma. There were 6 females and 10 males (average age 24.7 years). These patients received palliative radiotherapy and combination chemotherapy with vincristine, cyclophosphamide, doxorubicin, actinomycin D and dacarbazine. At 36 months, 3 patients are alive tumor-free and 1 patient is alive with stable disease. 12 patients died, 2 after temporarily achieving complete remission.

Abdominal Neoplasms↗

PubMed2004

Good outcome for infant of mother treated with chemotherapy for ewing sarcoma at 25 to 30 weeks' gestation.

There have been only three previous reports of women with Ewing sarcoma who received chemotherapy while pregnant. A 17-year-old woman with Ewing sarcoma was treated with a combination of doxorubicin (Adriamycin) and ifosfamide during the 25th to 30th week of gestation, and the baby was delivered at the 32nd week. The baby was developing normally at follow-up at 8 months of age. This case report supports the idea that in this situation, the pregnancy can be continued to await fetal growth, and second-trimester chemotherapy may not have a deleterious effect on the fetus.

Adolescent↗

PubMed1990

Use of MR imaging to assess results of chemotherapy for Ewing sarcoma.

MR imaging was used to monitor the results of initial chemotherapy of primary Ewing sarcoma of bone. The signal intensities of the soft-tissue and marrow components of the tumor were evaluated on T2-weighted images obtained in 10 patients (nine with responsive tumors) at presentation and during and immediately after completion of two cycles of chemotherapy. MR evidence of marrow and soft-tissue involvement was seen in all tumors at presentation. After treatment, the bone-marrow component of the nine drug-sensitive tumors showed an increase in signal intensity that in eight cases became comparable to that of water. Changes in signal intensity of the soft-tissue component were variable, consisting of increases in two of the responsive lesions, no change in three, a decrease in two, and complete resolution of the soft-tissue mass in two. There was no increase in signal intensity of either the bone-marrow or the soft-tissue component of the single nonresponsive tumor. All of the responsive tumors showed advanced healing, and abundant bony sclerosis was apparent on CT. Bone-marrow examinations, performed in seven of the nine patients with responsive lesions, disclosed no evidence of tumor in four. Two patients had residual extramedullary tumor; the nonresponsive lesion contained sheets of tumor cells. The increase in marrow signal intensity on T2-weighted images was associated with replacement of marrow elements by a loose, hypocellular myxoid matrix containing modest amounts of collagen, consistent with response to chemotherapy and eradication of disease. Therefore, an increase in the T2-weighted signal intensity of the bone-marrow component of Ewing sarcoma of bone reflected a favorable response to chemotherapy. MR signal changes, however, were not predictive of resolution of malignant disease within adjacent soft tissue.

Adolescent↗

PubMed1978

Ewing sarcoma of the mandible: report of case.

A case of Ewing sarcoma, primarily localized in the mandible in a 19-year-old woman, is reported. The case report covers the initial symptoms, which are often misleading, the history, clinical, radiographic, and histologic features; and the treatment by surgery complemented with radiotherapy and chemotherapy.

Adult↗

PubMedDate not supplied

Expression and regulation of the transcriptional repressor ZNF43 in Ewing sarcoma cells.

In vitro, cells derived from Ewing sarcoma (ES) with the characteristic somatic rearrangement between the genes EWS and FLII can be induced to differentiate toward a neuronal phenotype by exposure to agents such as dibutyryl cyclic AMP (db cAMP) or retinoic acid. Therefore, expression of the chimeric Ews-Flil protein does not irreversibly block the capacity of Ewing cells to engage in the neuronal differentiation program initiated by these agents. To identify genes that might be involved in the maintenance of Ewing cells in their undifferentiated state, a PCR-based differential display method was used to compare gene expression patterns in Ewing cell lines with those induced to differentiate toward a neuronal phenotype. A cDNA was expressed at high levels in proliferating Ewing-derived EW-1 cells and downregulated in EW-1 cells induced to differentiate, which corresponds to ZNF43, a multi-zinc finger protein containing the Krüppel-associated box (KRAB) transcriptional repression domain. Treatment of EW-1 cells with antisense oligonucleotides complementary to ZNF43 mRNA induces morphological differentiation and growth arrest. These findings suggest a role for ZNF43 in the maintenance of ES cells in an undifferentiated state, and that ZNF43 could be a primary target for differentiation stimuli in Ewing cells.

Blotting, Northern↗

PubMed1994

[Detection of EWS-/FLI-1 gene fusion transcripts by RT-PCR as a tool in the diagnosis of tumors of the Ewing sarcoma group].

Recent cloning of the chromosome breakpoint regions of the reciprocal chromosomal t(11;22) (q24;q12) has revealed that the breakpoints were localized within the EWS gene (Ewings sarcoma gene) on chromosome 22 and the FLI-1 gene on chromosome 11. Thus, molecular genetic techniques were applicable for the detection of this genetic aberration, which occurs as a consistent feature of the Ewings tumor family. By reverse transcription and polymerase chain reaction technique (RT-PCR) in 78% of Ewings sarcoma derived cell lines, and in 91% of primary Ewings tumor tissue t(11;22) specific EWS/FLI-1 fusion transcripts were detected. Furthermore, in bone marrow samples from an Ewings sarcoma patient contaminating tumor cells could be shown by RT-PCR. Our results indicate that molecular genetic detection of the t(11;22) translocation opens a new modality for the differential diagnosis and the staging of Ewings tumor patients.

Base Sequence↗

PubMed2006

Localization of genetic elements of intact and derivative chromosome 11 and 22 territories in nuclei of Ewing sarcoma cells.

Recurring chromosomal abnormalities are associated with specific tumour types. The EWSR1 and FLI1 genes are involved in balanced translocation t(11;22)(q24;q12), which is present in more than 85% of Ewing sarcomas. In our previous study, we have found that the fusion genes pertaining to both derivative chromosomes 11 and 22 in Ewing sarcoma cell nuclei are shifted to the midway nuclear position between the native EWSR1 and FLI1 genes. In this contribution we focused our attention at nuclear positioning of other genetic elements of chromosomes 11 and 22 in order to find if the whole derivative chromosomes or only their translocated parts change their nuclear positions in comparison with the native chromosomes. Using repeated fluorescence in situ hybridization and high-resolution cytometry, 2D radial positions of EWSR1, BCR, FLI1, BCL1 genes and fluorescence weight centres of chromosome territories were compared for intact and derivative chromosomes 11 and 22 in nuclei of three Ewing sarcoma samples. Significant radial shift was obtained for the derivative EWSR1, FLI1 and BCL1 genes and for the derivative chromosome 11 compared with the intact ones and not very significant for chromosome 22 and the BCR gene. Our results also suggest that the mean nuclear positions of fusion genes are determined by the final structure of the derivative chromosomes and do not depend on the location of the translocation event.

Calmodulin-Binding Proteins↗

PubMed1994

Periosteal Ewing sarcoma.

PURPOSE: To evaluate the imaging and histopathologic findings and clinical course of patients with periosteal Ewing sarcoma (PES). MATERIALS AND METHODS: Conventional radiographs, computed tomographic (CT) scans, and magnetic resonance (MR) images in 10 adolescents and one adult were evaluated for the extent and character of PES. RESULTS: Ten of 11 masses were in the proximal extremities and one in the fibula; nine were diaphyseal and two, metadiaphyseal. Radiographs and CT scans showed a subperiosteal mass that did not invade the medullary cavity, was contiguous with and elevated the periosteum, and produced a Codman triangle and periosteal reaction. No PES exhibited osseous or cartilaginous matrix calcifications. MR imaging and histopathologic examination helped confirm the sparing of cancellous bone and the subperiosteal location. CONCLUSION: PES differs from the more common medullary and soft-tissue Ewing sarcomas in location, marked male predominance, and lack of presenting metastases. Except for the absence of matrix calcifications, PES resembles other periosteal sarcomas in imaging characteristics and a less aggressive clinical course.

Adolescent↗

PubMed2006

High histologic and overall response to dose intensification of ifosfamide, carboplatin, and etoposide with cyclophosphamide, doxorubicin, and vincristine in patients with high-risk Ewing sarcoma family tumors: the Bambino Gesù Children's Hospital experience.

BACKGROUND: Ewing sarcoma (ES) and extraosseous ES/primitive neuroectodermal tumors (PNET) share histopathologic features of the ES family of tumors (ESFT). The authors report on their results from a regimen of ifosfamide, carboplatin, and etoposide (ICE) with cyclophosphamide, doxorubicin, and vincristine (CAV) dose intensification in patients with high-risk ESFT. METHODS: Since 1990, patients with ESFT and with 1 or more of the following risk factors were reviewed: tumor volume > 200 mL, tumor site with a poor prognosis, and pulmonary and/or bone marrow metastases. RESULTS: Thirty-six patients with ESFT who were involved in the study were divided into 2 arms of 18 patients each. One group received treatment with various regimens, and the other group received treatment with ICE plus CAV. The disease was brought under control more rapidly in the latter patients, for whom surgery was more easily feasible, and up to 90% of patients achieved a major response, with an estimated 3-year overall survival rate of 67% +/- 12%. CONCLUSIONS: The current results showed that ICE plus CAV was tolerated well and was effective in the studied subset of tumors, indicating that dose intensification correlates with better disease control, a high percentage of necrosis, and conservative surgery in patients with high-risk ESFT.

Adolescent↗

PubMed2004

Efficacy of combined surgery and irradiation for localized Ewings sarcoma family of tumors.

BACKGROUND: Local tumor control for patients with Ewings sarcoma family of tumors (ESFT) is favorable when the primary tumor is resectable or small. Local failure rates for patients who received combined surgery and RT were reviewed to determine outcome and prognostic factors. PROCEDURES: We performed a retrospective review of all patients with localized ESFT treated with combined definitive surgery and RT between 1978 and 2001, at St. Jude Children's Research Hospital. Rates of local failure, survival, and prognostic factors for recurrence were determined in 39 patients who received combined local therapy. Systemic treatment included vincristine, actinomycin D, cyclophosphamide, and doxorubicin (6 patients), with the addition of ifosfamide and etoposide (33 patients). RESULTS: With a median follow-up of 8.7 years the 5- and 8-year survival estimates were 89.2 +/- 5.4% and 82.8 +/- 7.3%. The 8-year incidence of local failure was 10.8 +/- 5.2%. The 8-year local failure rate for patients with positive surgical margins was 17% and for negative surgical margins 5% (P = 0.25). Overall survival was improved for patients with negative surgical margins (94 vs. 71%, P = 0.052). Tumor size, site, histologic response, and radiation dose did not significantly alter the rate of local failure. CONCLUSIONS: Patients with ESFT managed with definitive surgery and irradiation have favorable local control rates. Even among patients with unfavorable prognostic factors local tumor control remained excellent.

Adolescent↗

PubMed1983

Treated Ewing sarcoma: radiographic appearance in response, recurrence, and new primaries.

Healing of Ewing sarcoma after therapy has a predictable radiographic appearance, eventually stabilizing and remaining constant in appearance. This study is based on 35 patients in whom adequate follow-up radiographs were available. The expected sequence was evident in 21 patients. Complete healing was evident in about 1 year. Failure to heal as expected or lytic change in healed bone indicated recurrent or refractory disease in 11 patients who developed distant metastases or clinical signs of recrudescence at the primary site. This complication was evident within months to 2 years of the initial treatment. In three patients, development of a new, primary, osteogenic sarcoma was heralded by disorganized tumor new bone in an osteoid matrix, evident only after several years of stability. These new osseous malignancies are probably treatment-related.

Adolescent↗

PubMed1996

Monitoring neovascularity as an indicator to response to chemotherapy in osteogenic and Ewing sarcoma using magnetic resonance angiography.

Histologic studies on resected specimen have shown that tumor neovascularity is related to prognosis and response to therapy in a variety of human neoplasms. In nine patients with osteogenic or Ewing sarcoma, we evaluated the use of magnetic resonance angiography (MRA) to assess neovascularity non-invasively in vivo and to monitor response to chemotherapy. Seven patients with osteosarcoma and two patients with Ewing sarcoma were studied before and after chemotherapy by MRA (2-D time-of-flight gradient-echo sequence, TR = 50 msec, TE = 9.5 msec, theta = 50 degrees, acquisition time 13 min). MR angiograms were assessed for chemotherapy-induced changes in neovascularity. MRA showed both feeder vessels and neovascularity. Six patients responded to chemotherapy ( > or = 90% histologic tumor necrosis). MRA demonstrated marked reduction in neovascularity in all responders. Three patients did not respond to chemotherapy ( < 90% histologic tumor necrosis). MRA demonstrated persistent or increased neovascularity in the non-responders. MRA provides a unique opportunity to study tumoral neovascularity noninvasively in vivo and helps to assess response to chemotherapy in patients with osteogenic or Ewing sarcoma. These general principles may be applicable to other human tumors.

Adolescent↗