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A case of Ewing sarcoma in a girl 13 year-old with multiple bone and soft tissue lesions at the time of diagnosis is presented. The lungs were free from the disease. This patient represents most likely a simultaneous (synchronous) Ewing sarcoma.
Two cases of Ewing sarcoma were karyotyped by using a fluorodeoxyuridine synchronisation procedure on short term cultures of fresh tumour material. This procedure enables rapid cytogenetic analysis of such material. Both showed relatively simple karyotypes, 48,XX, +2, +7,t(11;22)(q24;q12), ?dup(12)(q21----q24) and 47,XX,i(1q),t(11;22)(q24;q12). These results further support this translocation between chromosomes 11 and 22 as a specific marker for Ewing sarcoma. Cytogenetic studies in such cases are an important adjunct to histological studies and in some cases may contribute to the resolution of the diagnosis.
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Ewing sarcoma is a relatively uncommon malignant bone neoplasm that usually occurs in children and young adults and involves the major long bones, pelvis, and ribs. Primary diaphragmatic Ewing sarcoma is extremely rare. To the best of our knowledge, only three cases of primary Ewing sarcoma of the diaphragm have been reported. A 12-year-old girl presented spontaneous occurrences of the right hemothorax. After drainage, a roentgenogram film, computed tomography, ultrasonography, and magnetic resonance image showed a giant mass on the right diaphragm. Primary diaphragmatic tumor was resected totally by right posterolateral thoracotomy, and histologically, an extraskeletal Ewing sarcoma was identified. The patient received adjuvant radio-chemotherapy, and there was no evidence of disease 10 months after the operation. Although extremely rare, extraskeletal Ewing sarcoma should be kept in mind in the differential diagnosis of diaphragmatic soft tissue tumors.
Ewings' sarcoma involving the facial bones is rare although it is the second most frequent bone malignancy. For the diagnosis a biopsy is needed. Currently the treatment is the combination of chemotherapy and radiotherapy with surgery indicated in only a few instances. We report a case of Ewing's sarcoma of the mandible and describe its clinicopathologic features and the treatment of this disease.
BACKGROUND: The time between the initial symptoms of osteosarcoma and Ewing sarcoma and the correct diagnosis and treatment is long. Over the last two decades, the prognosis for patients with these diseases has dramatically improved due to a new chemotherapy regimen. As a consequence, a limb-sparing operation has become an alternative to amputation. The aim of this study was to establish the initial symptoms and physical signs of osteosarcoma and Ewing sarcoma from the records of the first medical visit and to identify early characteristics of the diseases to shorten the delay to diagnosis. METHODS: A group of patients with osteosarcoma or Ewing sarcoma was identified from the Swedish Cancer Register of patients thirty years old and younger. Records from the first medical visit due to symptoms related to the bone tumor were obtained for 102 patients with osteosarcoma and forty-seven patients with Ewing sarcoma. RESULTS: Pain related to strain was reported by eighty-seven (85 percent) of the patients with osteosarcoma and thirty (64 percent) of those with Ewing sarcoma, but only twenty-one (21 percent) of the patients with osteosarcoma and nine (19 percent) of those with Ewing sarcoma reported pain at night. Forty-eight (47 percent) of the patients with osteosarcoma and twelve (26 percent) of those with Ewing sarcoma related the onset of symptoms to minor trauma occurring around the same time. A palpable mass was noted in forty (39 percent) of the patients with osteosarcoma and sixteen (34 percent) of those with Ewing sarcoma at the first visit, and in most cases the tumor diagnosis was suspected. There was a broad spectrum of misdiagnoses; the most common was tendinitis, which was the initial diagnosis in thirty-two (31 percent) of the patients with osteosarcoma and ten (21 percent) of those with Ewing sarcoma. The doctor's delay (the period from the first medical visit due to the symptoms to the correct diagnosis) was longer for Ewing sarcoma than for osteosarcoma (nineteen weeks and nine weeks, respectively; p < 0.0001). CONCLUSIONS: An initial symptom of both osteosarcoma and Ewing sarcoma was pain, which was intermittent and often related to strain but not frequently felt at night. A history of trauma was common, but the clinical course often diverged from what was expected from trauma. The clinical course of osteosarcoma and particularly of Ewing sarcoma was not steadily progressive but intermittent, which often misled the doctor into believing that the condition was temporary. The most important clinical feature was a palpable mass, which was noted in more than one-third of the patients at the first visit. This finding emphasizes that a thorough physical examination is absolutely necessary.
Ewing sarcoma is a malignant tumor of bone that rarely spreads to the head and neck. We describe an unusual case of metastatic Ewing sarcoma involving the calvarium mimicking olfactory neuroblastoma, an uncommon neuroectodermal tumor of the anterior skull base. Pertinent clinical, radiological, and pathologic features of these tumors are described, with particular attention to their imaging characteristics.
PURPOSE: Ewing sarcoma is a common pediatric bone tumor with an unfavorable prognosis for metastatic or recurrent disease. Cellular immunotherapy may provide new treatment options and depends on the cytolytic death receptor and perforin/granzyme pathways. Expression of death receptor pathway inhibitor cellular FLICE inhibitory protein (cFLIP), initiator caspase-8, and granzyme B inhibitor protease inhibitor-9 (PI-9) have been reported to determine susceptibility to cell- and chemotherapy-mediated killing in several tumor types. Here, we have studied their in vitro and in vivo expression in Ewing sarcoma and the implications for susceptibility to cytotoxicity. EXPERIMENTAL DESIGN: Ewing sarcoma cell lines (n = 8) were tested for cFLIP, PI-9, and caspase-8 expression. Functional significance was tested by anti-Fas antibody (death receptor pathway) or natural killer cell (perforin/granzyme pathway) treatment. Immunohistochemistry was done on 28 sections from 18 patients. In half of the cases, sequential material, including metastases, was available. RESULTS: Although all tested Ewing sarcoma cell lines expressed cFLIP, resistance to CD95/Fas-mediated apoptosis was only observed in two cell lines lacking caspase-8 expression. PI-9 was expressed at low levels in four of eight Ewing sarcoma cell lines, but positive cell lines remained susceptible to perforin/granzyme-mediated killing. In primary Ewing sarcoma, including metastases, cFLIP was abundantly expressed in 18 of 18 patients. Caspase-8 was expressed in all patients but showed more intertumoral and intratumoral variation in both intensity and heterogeneity of staining. PI-9, in contrast, was undetectable. CONCLUSIONS: The expression patterns of cFLIP, caspase-8, and the absence of PI-9 provide a rationale to preferentially exploit the perforin/granzyme pathway in cytotoxic therapies against Ewing sarcoma.
Ewing Sarcoma has been the second most common primary osseous malignancy in childhood and adolescence. It has been described as a highly aggressive neoplasm. This is the oldest case report in the literature with Ewing Sarcoma.
BACKGROUND: Local recurrence in Ewing sarcoma is associated with a poor prognosis. The purpose of the study was to determine the factors that predict local recurrence after surgical treatment of the primary tumor. METHODS: Between 1990 and 2001, 64 patients underwent surgical resection of Ewing sarcoma. Surgical margins were assessed histologically and radiologically. Response to preoperative chemotherapy was determined by detailed specimen mapping. Local recurrence-free survival (LRFS) was calculated by Kaplan-Meier analysis. Multivariate analysis was performed with the Cox proportional hazards model. RESULTS: A number of factors were found to be associated with local recurrence on univariate analysis. Patients with a good response to chemotherapy (> or = 90% tumor necrosis), had superior LRFS at 5 years (86% vs 51%, P = .015). Central site of disease was associated with an increased rate of recurrence. The LRFS at 5 years was 50% for the chest wall, 74% for pelvic/scapular, and 86% for extremity tumors (P = .083). Positive surgical margin was not a strong predictor of recurrence (P = .72). A critical analysis of minimal surgical margin based on preoperative magnetic resonance imaging (MRI) and computed tomography (CT) scans also failed to reveal an association between margin and local recurrence. In multivariate analysis, the 2 independent predictors of local recurrence were histological response to chemotherapy and central site of disease. CONCLUSION: Local recurrence after surgical resection is a complex phenomenon. An important predictive factor is the response to chemotherapy. In the current study, this seems to have the largest impact. Central site of disease may be a second independent predictive factor.
Radiological findings of Ewing sarcoma have been well documented, and bone expansion or cystic change is known to be a relatively uncommon finding. On the other hand, expected changes in Ewing sarcoma after treatment have also been reported, e.g. regression of extraosseous soft tissue mass, regression and organization of the periosteal reaction, and remodelling of the lytic or sclerotic bone changes. However, remodelling of bone is often limited or incomplete. We saw an atypical change in Ewing sarcoma after treatment due to lack of significant remodelling of the expanding bone. Because such changes might be a cause of misdiagnosis, knowledge of it will help the radiologist in the follow up evaluation.
Ewing sarcoma and related neoplasias are characterized by the presence of specific chromosomal translocations resulting in EWS/ETS gene rearrangements. Created EWS/ETS-oncogene fusion transcripts can be detected in up to 98% of ESFT and provide tumour-specific markers useful in diagnostics. Using RT-PCR for detection of this aberration we can reveal minimal amounts of tumour cells contaminating BM, blood or apheresis products. We have examined BM samples from 22 patients (21 newly diagnosed and one recurrent disease) with histologically confirmed ESFT for the presence of contaminating tumour cells in BM at the time of diagnosis. Sixteen patients presented with localized disease, six had distant metastases at the first presentation. Ewing sarcoma cells were detected in the BM of 5/16 (31%) patients with localized disease and 3/6 (50%) with clinically detectable metastases at diagnosis. BM smears prepared from the same aspirates evaluated by light microscopy were all negative, even in two patients with multiple bone disease. We have confirmed the high sensitivity of the RT-PCR assay for detection of minimal BM infiltration in localized and metastatic ESFT. We have found that more than a quarter of patients with localized ESFT have minimal BM infiltration. Although the clinical significance of the minimal disease detected at the molecular level remains unknown, RT-PCR evaluation may enable better stratification of patients into risk groups in the future.
Extraosseous Ewing sarcoma--a rare clinical entity--is described and the radiographic and histologic features discussed. Histologically this type of tumor may be differentiated from other soft tissue malignancies, but the ultrastructural glycogen content of the cytoplasm makes it frequently inseparable from the conventional osseous form of Ewing sarcoma. In spite of the presence of a huge paraosseal mass with bone erosion or periosteal new bone, computed tomography was the only preoperative modality able to provide evidence of non-involvement of the marrow cavity. However, histologic documentation is necessary to prove that one is dealing with a totally extraosseous Ewing sarcoma.
Ewing sarcoma (E.S.) is a rare malignant tumour of the bone, dubious in origin and uncertain in the direction of its differentiation [2,3,7,10]. Recent reports have presented description of primary neoplasms of soft tissues which revealed no differences from E.S. in light and electron-microscopy but had no connection with the skeleton [1,4,5,6,8,9]. These lesions, found in subcutaneous tissue of the trunk and limbs, in the retroperitoneum and in paravertebral regions were called extraskeletal Ewing sarcoma (E.E.S.) or soft tissue Ewing sarcoma. As tumours of this type are infrequent and may cause diagnostic difficulties, we shall present a recently diagnosed case of extraskeletal Ewing sarcoma.
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As a result of overlapping morphologic and immunohistochemical features, it can be difficult to distinguish synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma/primitive neuroectodermal tumor in core biopsies. To analyze and compare immunohistochemical profiles, we stained tissue microarrays of 23 synovial sarcomas, 23 malignant peripheral nerve sheath tumors, and 27 Ewing sarcomas with 22 antibodies potentially useful in the differential diagnosis, and analyzed the data with cluster analysis. Stain intensity was scored as none, weak, or strong. For CD99, tumors with membranous accentuation were independently categorized. Cluster analysis sorted five groups, with like tumors clustering together. Synovial sarcoma clustered into two groups: one cytokeratin and EMA positive (n = 11), the other mostly cytokeratin negative, EMA positive, bcl-2 positive and mostly CD56 positive (n = 9). Malignant peripheral nerve sheath tumor clustered into two groups: one S100 positive, with nestin and NGFR positivity in most (n = 10), the other mostly S100 negative, and variably but mostly weakly positive for nestin and NGFR (n = 11). Ewing sarcomas clustered into a single group driven by membranous CD99 staining. Thirteen cases failed to cluster (outliers), while three Ewing sarcomas clustered into groups of other tumor types. Paired antibodies for each tumor type determined by visual assessment of cluster analysis data and statistical calculations of specificity, sensitivity, and predictive values showed that EMA/CK7 for synovial sarcoma, nestin/S100 for malignant peripheral nerve sheath tumor, and membranous CD99/Fli-1 for Ewing sarcoma yielded high specificity and positive predictive values. Cluster analysis also highlighted aberrant staining reactions and diagnostic pitfalls in these tumors. Hierarchical cluster analysis is an effective method for analyzing high-volume immunohistochemical data.
STUDY DESIGN: A case study of a patient with Ewing sarcoma of T8 and T9 with paravertebral and chest wall involvement, who underwent neoadjuvant chemotherapy and subsequent multilevel en bloc spondylectomy and chest wall excision using a simultaneous anterior and posterior approach. OBJECTIVE: To show the feasibility of treating Ewing sarcoma of the thoracic spine with paravertebral and chest wall extension by multiagent chemotherapy followed by a multilevel en bloc spondylectomy and chest wall excision using a simultaneous anterior and posterior approach. SUMMARY OF BACKGROUND DATA: Ewing sarcoma is a primary malignant bone tumor that occasionally involves the spinal column. Most patients with Ewing sarcoma of the spine are treated with systemic chemotherapy followed by definitive local control. Radiation therapy is the usual mode of local control in these patients because the spinal column has historically been considered a surgically inaccessible site where wide surgical margins are difficult to obtain. However, en bloc spondylectomy techniques have been described that can probably further decrease the risk of local recurrence, thereby minimizing or even eliminating the need for radiation therapy. To our knowledge, a combined en bloc spondylectomy and chest wall excision in a patient with Ewing sarcoma in the spine has not been previously reported. METHODS: Neoadjuvant chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide was administered. After completion of the chemotherapy, an en bloc spondylectomy of T8 and T9 with removal of the chest wall was achieved using a simultaneous anterior and posterior approach to the spine. A stackable carbon fiber cage filled with autograft and allograft bone was inserted between T7 and T10. The spine was stabilized with anterior and posterior instrumentation. The chest wall was reconstructed with contoured polymethylmethacrylate and polypropylene (Marlex, Textile Development Associates, Inc., Franklin Square, NY) mesh. RESULTS: The patient maintained normal neurologic function, and pain was lessened. The margins were free of tumor, and tumor necrosis was 100%. After surgery, radiotherapy was not administered. No local tumor recurrence or distant metastases were evident at the last follow-up. Balance in the coronal and sagittal planes was maintained. The patient has returned to work and resumed normal activities of daily living. CONCLUSIONS: Multilevel en bloc spondylectomy and chest wall excision performed using a simultaneous anterior and posterior approach is a safe and effective technique that may be used to achieve adequate margins in select patients with malignant tumors involving the thoracic spine and chest wall. This technique can eliminate the need for radiation therapy in patients with Ewing sarcoma and probably decreases the risk of local recurrence compared with radiation therapy alone.
Extraskeletal Ewings sarcoma is a tumour of neuroectodermal origin sharing close similarities with Ewings sarcoma of bone. We report the case of a 21 year old 16 week pregnant woman presenting with vomiting and weight loss and found to have an extraskeletal Ewings sarcoma of the small bowel. In a review of the literature there are no previous reports of extraskeletal Ewings sarcoma occurring in the small bowel. The diagnosis of extraskeletal Ewings sarcoma and the complicated management of a young pregnant woman with a malignant tumour are described.