Osseous Ewing's Sarcoma Research Articles

A large femoral primary cutaneous Ewing's sarcoma in a 35-year-old woman: Case report.

IntroductionExtraskeletal Ewing's sarcoma is a rare malignant tumor of mesenchymal origin, which is histologically similar to primary osseous Ewing's sarcoma. It has been well described in deep soft tissues. However, location in cutaneous or subcutaneous tissue has rarely been reported. Being seen principally in children, it can be seen, rarely, in old men.Case presentationWe present a case of large primary cutaneous Ewing sarcoma within the left thigh of a 35-year-old woman, without osseous involvement. Physical examination. Histologically, it was a small round cell tumor that marked strongly for CD99. The diagnosis of cutaneous Ewing sarcoma was performed.DiscussionThe things that distinguish our case are that it is the first case in Syria, in addition to the size of the large tumor with diameters of 15 × 20 cm, and it is in a 35-year-old woman.ConclusionEwing sarcoma is a rare malignant small round cell tumor of the skin and subcutaneous tissue. It should be differentiated from other cutaneous neoplasms composed of small round cells.

A Comparison of Osseous and Extraosseous Ewing Sarcoma.

To compare the clinicopathological characteristics, treatment responses, survival analysis of osseous Ewing sarcoma (OES) and extraosseous ES (EES). Observational study. Ankara City Hospital and Ankara Numune Training Research Hospital Medical Oncology Clinics from January 2005 to February 2020. Clinicopathological characteristics of histologically confirmed ES/PNET and followed up, and treatment modalities were recorded from patients' registration data-base of the hospital. Lactate dehydrogenase (LDH), alkaline phosphatase (ALP), hemoglobin were measured before chemotherapy or surgery. The patients with a second cancer, gall bladder/biliary tract diseases, viral hepatitis and other bone diseases were excluded. Sixty seven patients evaluated retrospectively. Out of the total patients, 56.7% consisted of OES, and 43.3% consisted of EES. The median age of the EES group (26 years) was significantly higher than that of the OES group (22 years, p = 0.008). The most common metastasis region was lung in both the groups. Age, LDH levels and stage of the disease were found to be statistically significant prognostic factors in univariate and multivariate analysis. The median OS of patients who started with local treatment (surgical, surgical ± radiotherapy) and followed up with chemotherapy was 82.6 months (95% CI, 55.2-110.1), while the median OS of patients who received local treatment between or after chemotherapy was 43.4 months (95% CI, 13.2-73.6, p = 0.042). Patients with extrosseus ES were significantly older. Age, LDH levels, stage of disease, local treatment followed by systemic therapy are important associated factors. Key Words: Osseous ewing sarcoma, Extraosseous ewing sarcoma, Chemotherapy, Local treatment.

Prognostic factors for survival in patients with Ewing's sarcoma using the surveillance, epidemiology, and end results (SEER) program database

BackgroundThe current study aims to determine cause-specific survival in patients with Ewing's sarcoma while reporting clinical risk factors for survival. MethodsThe Surveillance, Epidemiology, and End Results (SEER) Program database was used to identify patients with osseous Ewing's sarcoma from 1991 to 2010. Patient, tumor, and socioeconomic variables were analyzed to determine prognostic factors for survival. ResultsThere were 1163 patients with Ewing's sarcoma identified in the SEER Program database. The 10-year cause-specific survival for patients with non-metastatic disease at diagnosis was 66.8% and 28.1% for patients with metastatic disease. Black patients demonstrated reduced survival at 10 years with an increased frequency of metastatic disease at diagnosis as compared to patients of other race, while Hispanic patients more frequently presented with tumor size>10cm. Univariate analysis revealed that metastatic disease at presentation, tumor size>10cm, axial tumor location, patient age≥20 years, black race, and male sex were associated with decreased cause-specific survival at 10 years. Metastatic disease at presentation, axial tumor location, tumor size>10cm, and age≥20 years remained significant in the multivariate analysis. ConclusionsPatients with Ewing's sarcoma have decreased cause-specific survival at 10 years when metastatic at presentation, axial tumor location, tumor size>10cm, and patient age≥20 years.

From the Radiologic Pathology Archives: Ewing Sarcoma Family of Tumors: Radiologic-Pathologic Correlation

The Ewing sarcoma family of tumors includes osseous Ewing sarcoma, extraskeletal Ewing sarcoma, primitive neuroectodermal tumor, and Askin tumor. They share a karyotype abnormality with translocation involving chromosomes 11 and 22. Histologically, these lesions demonstrate crowded sheets of small round blue cells. Imaging features of osseous Ewing sarcoma often suggest the diagnosis, with aggressive long-bone destruction in the metadiaphysis of an adolescent or young adult and an associated soft-tissue mass. Focal areas of cortical destruction are frequent, allowing continuity between the intraosseous and extraosseous components. This continuity is also commonly seen as subtle channels extending through the cortex at computed tomography or magnetic resonance (MR) imaging, a finding that reflects the underlying pathologic appearance. Extraskeletal Ewing sarcoma commonly demonstrates a nonspecific radiologic appearance of a large soft-tissue mass affecting the paraspinal region or lower extremity. Askin tumor represents extraskeletal Ewing sarcoma involving the chest wall. Imaging typically reveals a large pleural-based mass and associated pleural effusion. Treatment of these tumors is usually a combination of neoadjuvant chemotherapy followed by surgical resection, which may be supplemented with radiation therapy. Imaging, particularly MR, is also vital to evaluate response to neoadjuvant therapy, direct surgical resection, and detect local recurrence or metastatic disease.

CT and Positron Emission Tomography/CT Findings of Mediastinal Extraskeletal Ewing's Sarcoma with Extensive Distant Metastasis: A Case Report

Extraskeletal Ewing’s sarcoma (EES) is a rare malignant small round cell tumor of neural crest origin, which is histologically similar to the more common osseous Ewing sarcoma. Different from the osseous counterpart, it shows a wider age presentation, preferentially occurring in children and young adults, younger than 30 years (1, 2). The most frequent sites of occurrence are the chest wall, lower extremities and paravertebral region, but it rarely occurs in the mediastinum. The recently reported CT finding of mediastinal EES is a large, non-calcified mass with heterogeneous enhancement, frequently infiltrating to the adjacent tissues. In spite of its aggressive behavior, distant metastasis is rare (1, 3). Although positron emission tomography (PET)/CT findings of EES have been rarely reported, it has been reported to show relatively weak fluorodeoxyglucose (FDG) uptake, considering the growth pattern (4). We report a case of a 68-year-old man diagnosed as mediastinal EES with extensive hematogenous and lymph node metastasis by a CT and PET/CT.

Metastatic Ewing's sarcoma/PNET of kidney in 40 year old patient

IntroductionPrimary renal Ewing sarcoma/PNET is an uncommon and very aggressive tumor. Presentation of case: We report the case of a young woman who underwent nephrectomy for a renal mass from unknown etiology. Histologic analysis found small tumoral cells in rosette formation, and immunohistochemical staining was positive for CD99, and focally positive for vimentin and Protein S-100. A post operative abdominal computed tomography (CT) scan revealed a residual renal processus with hepatic wounds and abdominal metastatic nodes.After 6 cycles of chemotherapy including Vincristine, Doxorubicin, Cyclophosphamide, the response was considered as good. DiscussionEwing's sarcoma/PNET of kidney is a member of the family of small round cell tumors and it should be differentiated from Wilms tumor, neuroblastoma, rhabdomyosarcoma and lymphoblastic lymphoma. The principle management of its treatment have been extrapolated from the treatment of osseous Ewing sarcoma of bone. ConclusionDespite aggressive treatment, primary renal Ewing sarcoma/PNET has a poor prognosis. It requires a multidisciplinary approach including oncologists, urologists and radiation oncologists.

Primary Intradural Extraosseous Ewing Sarcoma of the Spine: Case Report and Literature Review

To report a rare case of spinal intradural extraosseous Ewing sarcoma in an adult and review current literature. Although Ewing sarcoma belongs to the family, the treatment modalities are different, and thus the correct diagnosis is very important despite its rare occurrence. A 56-year-old woman presented with nocturnal bilateral buttock and leg pain. Magnetic resonance imaging (MRI) showed an enhancing intradural extramedullary extraosseous tumor at L1. A T12-L2 laminectomy was performed to resect the tumor. Immunohistochemical analysis confirmed the diagnosis of Ewing sarcoma. A thorough diagnostic workup did not reveal any bony origin of the tumor. Primary intradural central nervous system Ewing sarcoma is infrequently encountered and shares imaging and histopathological features with central primitive neuroectodermal tumors. Establishment of the right diagnosis is crucial because it mandates a distinct workup and treatment modality different from that for central primitive neuroectodermal tumor. Although osseous Ewing sarcoma predominantly occurs in children and young adults, extraosseous central nervous system Ewing sarcoma is not uncommon in adults and should therefore be considered in the differential diagnosis of extraosseous small blue cell tumors in adult patients.

Sarcome d’Ewing cutané primitif : difficultés diagnostiques et thérapeutiques

Ewing's sarcoma (or peripheral neuroectodermal tumour) is generally found in bone tissue, and a primary dermal site is extremely rare. We report a case of primary cutaneous Ewing's sarcoma in a 21-year-old woman. A 21-year-old woman presented with a scapular lesion that had been slowly developing for one year. The 1-cm lesion was removed and histological examination showed proliferation of small round cells in the dermis. Immunostaining revealed cytoplasmic membrane expression of CD99 and a negative immunoprofile for other small round-cell tumors. Ewing's sarcoma fusion gene transcripts were detected using fluorescence in situ hybridization (FISH). A staging examination revealed no other abnormalities. It was decided to treat the lesion as for osseous Ewing's sarcoma with wide resection followed by systemic adjuvant chemotherapy. Cutaneous Ewing's sarcoma raises concerns about diagnosis and treatment. Owing to the non-specificity of its clinical presentation, histology and immunoprofile, diagnosis of superficial Ewing's sarcoma is difficult and numerous differential diagnoses must be considered. When dealing with a surface tumour, the diagnosis of cutaneous Ewing's sarcoma must be considered. CD99 immunostaining and molecular testing for evidence of EWSR1 rearrangement are useful investigations to confirm the diagnosis. Furthermore, modalities of treatment must be carefully discussed. Cutaneous Ewing's sarcoma is currently treated in the same way as osseous Ewing's sarcoma (wide surgical excision, adjuvant radiotherapy when surgical margins are unsatisfactory, systemic adjuvant chemotherapy, and, in some cases, bone marrow transplant). However, some studies show a more favourable prognosis for cutaneous Ewing's sarcoma than for osseous Ewing's sarcoma. We may thus ask whether such aggressive multimodal treatment is needed.

Cervical extraskeletal Ewing's sarcoma: case report demonstrating radiological features and management

Extraskeletal Ewing's sarcoma is a rare tumour of mesenchymal origin, which is histologically similar to primary osseous Ewing's sarcoma. The majority of cases have been reported in the lower limb and paravertebral region, with a few cases reported in the neck. We report a patient with extraskeletal Ewing's sarcoma presenting with a right-sided neck mass, vocal fold palsy and T1 nerve root involvement. The detection of characteristic features on computed tomography and magnetic resonance imaging scans, in conjunction with histological analysis, was required to make the diagnosis. Radiological imaging demonstrated that the tumour was infiltrating through the neural exit foramina of the lower cervical nerve roots, with encasement of the vertebral artery. The patient was managed with combination chemotherapy for systemic control and irradiation for local control. Extraskeletal Ewing's sarcoma has a propensity to occur in a paravertebral location, being found rarely in the cervical region, and has a predilection to infiltrate through neural exit formina. Computed tomography and magnetic resonance imaging are helpful in the diagnosis of this rare tumour.

Ewing sarcoma: radiation dose and target volume.

The outcome of children and adolescents with Ewing sarcoma is impacted by many prognostic factors and often measured by estimates of: event-free, relapse-free, disease-free, or overall survival. However, the preferred assessment following radiation therapy is local control. A review of large group experiences over the past several decades was undertaken to assess the optimal radiation dose and volume for patients with localized, osseous Ewing sarcoma. New approaches and techniques to improve local control were also investigated. With multidisciplinary therapy, 5-year overall local control rates range from 58 to 93%. Following definitive irradiation, they are 53-86%. Recommended radiation therapy doses are 55.8-60.0 Gy. In the postoperative setting, gross disease requires 55.8 Gy; microscopic disease requires 45 Gy. Altered fractionation schemes have not improved local control. The appropriate irradiated volume is an involved field to the pretreatment tumor volume plus 2.0-2.5 cm margin, followed by a boost to the post-induction chemotherapy tumor volume with margin. Good radiation quality control with central review improves local control. Use of an involved radiation field requires accuracy in defining tumor volume. Techniques to improve local control include risk-adapted multidisciplinary therapy, intraoperative boost radiation, and high radiation doses as delivered by 3-dimensional conformal radiation. Intensity modulated and proton beam radiotherapy may offer an advantage at special sites. Innovative uses of radiation in the multidisciplinary setting will continue to provide excellent local control, improved function, and quality of life for young patients with localized Ewing sarcoma of bone.

Surgical management of some unusual Parapharyngeal space Tumors

We present out experience with the surgical management of 8 cases parapharyngeal tumors which were successfully operated using various approaches. Most tumors were quite large and presented special surgical challenges to safegaurd important structures and ensure complete tumors excision. Included in the series are 3 rare tumors, a myoepethilioma of the deep lobe of parotid, a Glomus intravagale tumor and a case of extra osseous Ewing's sarcoma. Depending on tumors location and extent, the transcervical, transparotid and midline mandibulotomy approaches were used and the techniques are described. All patients are currently doing well without any evidence of disease.

Treatment of non metastatic peripheral primitive neuroectodermal tumour (PNET)/Extra osseous Ewing's sarcoma (EOES): experience in the SIOP MMT 89 study

Treatment of non metastatic peripheral primitive neuroectodermal tumour (PNET)/Extra osseous Ewing's sarcoma (EOES): experience in the SIOP MMT 89 study

Retrospective analysis of ploidy in primary osseous and extraosseous Ewing family tumors in children.

To retrospectively study the DNA content in a series of childhood Ewing Family Tumors (EFT), and to investigate its prognostic value. The study was performed on a series of 27 EFTs (osseous Ewing's sarcoma, 18 cases; extraosseous Ewing's sarcoma, 2; peripheral neuroepithelioma, 4; Askin Rosai tumors, 3). Ploidy was investigated using both flow cytometry (FCM) and image cytometry (ICM) on tumor cell suspensions from formalin-fixed paraffin-embedded specimens or fresh frozen tissue obtained from the primary tumor at diagnosis. Ploidy was evaluable by FCM in all cases, and by ICM in 23/27. When fresh frozen tissue and paraffin-embedded samples from the same tumor were available for analysis, they yielded equal results. The rate of agreement between FCM and ICM was 82%. The majority of cases were diploid, and in the present series aneuploidy seemed to be associated with a poor outcome. These results suggest that aneuploidy could be an indicator of a bad prognosis in EFT; however, the small number of cases precludes any conclusion of statistical value. Larger retrospective studies on ploidy using archival material could be performed and their reliability is supported by the concordance of results from fresh and formalin-fixed tissue.

MR imaging findings in recurrent primary osseous Ewing sarcoma

The objective of this study was to determine the value of magnetic resonance (MR) imaging in diagnosing local recurrence of Ewing sarcoma. We retrospectively reviewed radiographs, Tc 99m-methylene diphosphonate (MDP) skeletal scintigraphy, computed tomography scans, and MR studies of 11 patients who had local recurrences of osseous Ewing sarcoma following initial responses to chemotherapy and local radiation. The MR images were compared to those of a control group of nine patients who had no evidence of relapse. T 1- and T 2-weighted MR images identified 9 of the 11 recurrences. Computed tomography was diagnostic in 4 of 6 cases evaluated, Tc 99m-MDP bone scintigraphy in 4 of 11 cases, and plain radiographs in 2 of 10. MR findings at relapse included changes in signal intensity, increased extent of abnormal marrow signal on T 1- and T 2-weighted images, and identification of a new soft tissue mass. These findings suggest that MR imaging is valuable in the routine follow-up of parimary osseous Ewing sarcoma.

Retroperitoneal extraosseous ewing's sarcoma with renal involvement: US and MRI findings

A case of retroperitoneal extraosseous Ewing's sarcoma (EES) with renal involvement, which simulated an exophytic renal mass, is reported. EES is a rare soft tissue tumor that can occur anywhere in the soft tissues, but is seen most commonly in the extremities. Although EES is histologically similar to osseous Ewing's sarcoma, it is usually found in older patients. EES should be included in the differential diagnosis of soft tissue tumors, especially in the second and third decade of life.

Ewing's sarcoma: Local tumor control and patterns of failure following limited-volume radiation therapy

Sixty children with localized osseous Ewing's sarcoma were treated between 1978 and 1988 with induction chemotherapy (cyclophosphamide, adriamycin), irradiation and/or surgery, and 10 months of maintenance chemotherapy (cyclophosphamide, adriamycin, dactinomycin, vincristine). Following induction chemotherapy, 43 patients received primary radiation therapy to limited radiation volumes defined by post-chemotherapy residual soft tissue tumor extension and initial osseous tumor extent. Irradiation was defined as low dose at 30–36 Gy (median 35 Gy) for 31 cases with objective response to induction chemotherapy and high dose at 50–60 Gy (median 50.4 Gy) for 12 patients with poor response to induction chemotherapy or with tumors ≥8 cm. Overall event-free survival at 5 years is 59% and local tumor control is 68%. Initial failures have been local (12), simultaneous local and distant failures (7), and distant (6). In the surgical resection group, 14 patients had complete resection without radiation therapy, and 3 patients had microscopic residual plus 35–41 Gy; 100% local control has been maintained. In 43 patients with primary radiation therapy group, local tumor control is 58% ( p = .004). Despite limited radiation volume, 18/19 local failures occurred centrally within the bone, well within the radiation volume. Imaging response to induction chemotherapy predicted local tumor control in the radiation therapy group: 62% with complete response/partial response versus 17% with no response/progressive disease ( p < 0.01). Local tumor control related strongly to primary tumor size in the radiation therapy group; among 31 cases receiving 35 Gy, local tumor control is 90% for lesions <8 cm versus 52% for tumors ≥8 cm ( p = .054). The central pattern of local failure in this experience suggests the effectiveness of limited radiation volume. The overall local tumor control rate following the tested dose level of 35 Gy appears to be inadequate, although results in selected cases with tumors 〈8 cm in greatest tumor dimension indicate potential efficacy in a yet limited experience.

Ewing??s Sarcoma

Advances in the treatment of Ewing's sarcoma have been dramatic. Present treatment protocols control local disease by radiotherapy, surgery, or both; systemic spread is limited by aggressive multiagent chemotherapy. In patients with localized osseous Ewing's sarcoma, five-year survival rates now range from 54% to an estimated 74%. With late relapse not uncommon, control of the primary lesion is critical to long-term survival. Several studies now show improved local control and possibly improved survival of patients with surgical treatment of primary osseous Ewing's sarcoma.

Prognostic value of histopathology in ewing's sarcoma. Long-term follow-up of distal extremity primary tumors

The pathologic material from 56 patients diagnosed initially as Ewing's sarcoma of the distal extremity and treated on National Cancer Institute protocols between 1968 and 1984 was reviewed and correlated with clinical outcome. Histologically, the tumors were categorized, based on recent pathologic criteria, into three diagnostic groups: (1) typical Ewing's sarcoma, (2) atypical Ewing's sarcoma, and (3) other (predominantly peripheral neuroepithelioma [PN]). Thirty-two patients (57%) had typical Ewing's, 13 (23%) were atypical, and 11 (20%) were in the "other" diagnostic category (seven [13%] PN, two primitive rhabdomyosarcoma, one primitive sarcoma of bone, and one synovial cell sarcoma). No cases of metastatic neuroblastoma, osteosarcoma, or lymphoma were found. Forty-five patients had localized disease at diagnosis; 11 had metastases. Patients with typical Ewing's sarcoma were less likely to have metastatic disease at the time of diagnosis. Only two of 32 patients with typical Ewing's sarcoma had metastases compared with nine of 24 patients in the two other groups. The pattern of relapse was also different in these other groups compared with typical Ewing's patients; five patients developed lymph node metastases and two patients developed brain metastases. Although the presence of metastatic disease at diagnosis was a strong negative prognostic factor, our histologic grouping was independently prognostic of clinical outcome in patients with localized disease. Patients with typical osseous Ewing's sarcoma had an improved overall survival (P2 = 0.03) and patients with other tumors (neither typical nor atypical Ewing's sarcoma) had a poorer disease-free survival (P2 = 0.02). Since no generally accepted histopathologic prognostic criteria exist for Ewing's sarcoma, the potential value of our proposed classification should be evaluated in a larger retrospective and a prospective study.

Peripheral primitive neuroectodermal tumor (peripheral neuroepithelioma) in children. A review of the St. Jude experience and controversies in diagnosis and management

All patients diagnosed with primitive neuroectodermal tumor (PNET) and extraosseous Ewing's sarcoma in one institution between 1962 and 1987 were reviewed. Of the 26 cases studied, 16 had been diagnosed originally as PNETs, seven tumors were rediagnosed as PNET or EOE by histologic review, and three tumors had an original diagnosis of extraosseous Ewing's sarcoma. To determine whether these diagnoses determine a group of tumors with unique biologic behavior and identifiable pathologic characteristics, clinical and treatment response data were compiled, and electron microscopic and immunohistochemical studies were done for those patients with adequate samples. With combined modality therapy, this group achieved a substantially shorter disease control interval than patients with disseminated osseous Ewing's sarcoma or disseminated neuroblastoma--10.8 months versus 17 months and 16 months, respectively. The pattern of relapse and distant spread also differed among these tumor types. Immunohistochemical studies (for example, neuron-specific enolase and beta 2 microglobulin) were helpful in confirming the diagnosis but were not definitive in themselves. Tentative diagnostic criteria are proposed for use in studies designed to provide further information on the nature and treatment of PNET. Some of the controversies regarding diagnosis are discussed. The authors propose a uniform approach to treatment of extraosseous Ewing's sarcoma and PNET in order to try to clarify their relation.