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PubMed2005

[Primitive neuroectodermal tumor/extraosseus Ewing sarcoma of the temporal bone].

The term primitive neuroectodermal tumor (PNET) should be included in the differential diagnosis of small round blue cell tumors, which come from the neural crest and explain their neuroectodermal character. This is one of the most indifferentiated histological types of malignant tumors. In the PNET family, we high light the Ewing's Sarcoma, that is frecuently located in bony sites and has a less frequent localization in soft tissues. We focus on one atypic case of PNET/Extraosseus Ewing's Sarcoma that involves the external auditory canal and infiltrates the temporal bone. Surgery followed by postoperative chemotherapy and radiation offers the best local control of the disease.

Adult↗
PubMed1995

Ewings sarcoma as an etiology for persistent back pain in a 17-year-old girl after trauma to the back.

Back pain--a common complaint familiar to all physicians--can be either a manifestation of musculoskeletal dysfunction or a symptom of a more serious underlying disease. Unfortunately, the diagnosis can be difficult, and back pain that is not resolved by conservative treatment requires aggressive investigation. The author presents the case history of a 17-year-old patient whose back pain had been extensively evaluated by a series of specialists to no avail until further workup revealed the problem to be Ewing's sarcoma. The steps required to diagnose elusive back pain are presented, together with a brief discussion of cauda equina syndrome and conus medullaris syndrome, components of both of which were found in this patient.

Adolescent↗
PubMedDate not supplied

[Local control in Ewing sarcoma. Comparative results following intralesional, marginal and tumor resection with healthy bone].

According to the results obtained so far in the CESS studies there is evidently no difference, as regards the frequency of local recurrence, between patients who have been surgically treated only and those who have had surgery and subsequent radiation therapy, provided that Enneking's guidelines on tumor excision according to regions are adhered to. Generally speaking, the recommendation still applies that primary local treatment of Ewing's sarcoma should be surgical. Further follow-ups continue to be necessary to establish whether postoperative radiation can be dispensed with. Is has become clear that radiation therapy alone will result in equally good local control only with small Ewing's sarcomas which respond well to preoperative chemotherapy.

Bone Neoplasms↗
PubMed2001

Adamantinoma-like Ewing's sarcoma and Ewing's-like adamantinoma. The t(11; 22), t(21; 22) status.

Adamantinoma of the long bones and Ewing's sarcoma are two malignant tumours between which, at first sight, there seems to be no morphological and clinical relationship. Both tumours, however, are known to express cytokeratins. Adamantinoma is a tumour of true epithelial nature, predominantly expressing cytokeratins 14 and 19. Ewing's sarcoma, believed to be from neuroectodermal origin, like other mesenchymal tumours, can aberrantly express cytokeratin 8 and 18. In the literature there are some reports of tumours showing clinical and/or morphological overlap between adamantinoma and Ewing's sarcoma, suggesting a possible relationship. These studies are mostly based on the epithelioid configuration of these lesions and their cytokeratin expression on immunohistochemistry. This raises the question of whether there is occasionally a morphological similarity between adamantinoma and Ewing's sarcoma, or whether there is a common genetic background. The Ewing's sarcoma/primitive peripheral neuroectodermal tumour (PNET) family is characterized in 90-95% of cases by a t(11; 22) and in 5-10% of cases by t(21; 22). In the few reports in the literature on cytogenetic investigations on adamantinoma, these translocations were never found using classical karyotyping. This study investigated the putative presence of t(11; 22) and t(21; 22) in 14 cases of adamantinoma by RT-PCR. These translocations were not found in any of these cases. The results support the view that these tumours are genetically and clinically distinct, but may eventually show overlapping morphological and immunohistochemical features.

Ameloblastoma↗
PubMed1980

Solid dendritic cell angiosarcoma: re-interpretation of extraskeletal sarcoma resembling Ewing's sarcoma.

Twenty cases of a type of extraskeletal tumour resembling Ewing's sarcoma (Angervall and Enzinger, 1975) were studied. The tumour affected mainly soft tissues in a variety of sites. Throughout the long follow-up period, there was only one instance of non-recurrence after excision. Most of the tumours became generalised within 3 years. One case developed general spread after 13 years. The tumour shows predominantly expansive growth and a solid architecture which, in places changes to reticular or sinusoidal structures. The predominant dendritic tumour cells show multiple junction complexes. Weibel-Palade granules can occasionally be demonstrated in their cytoplasm. In foci of peritheliomatous pattern the tumour cells become elongated, contain an abundance of endoplasmic reticulum and give rise to pericellular basement membranes, although typical collagen was rarely found. Judged by its structural and cytological features the tumour is a form of angiosarcoma, mostly of the endothelial type but with pericytic variations. Since neither its cellular features nor its biological behaviour correspond to those of Ewing's sarcoma, a change of name is proposed.

Adolescent↗
PubMed2000

Ewing sarcoma of the rib: results of an intergroup study with analysis of outcome by timing of resection.

OBJECTIVE: We sought to establish the outcome and optimal therapeutic sequence for patients with nonmetastatic Ewing sarcoma/primitive neuroectodermal tumor of the chest wall. METHODS: Patients 30 years of age or younger with nonmetastatic Ewing sarcoma/primitive neuroectodermal tumor of the bone were randomly assigned to receive vincristine, doxorubicin, cyclophosphamide, and dactinomycin or those drugs alternating with ifosfamide and etoposide. Local control was obtained with an operation, radiotherapy, or both. RESULTS: Fifty-three (13.4%) of 393 patients had primary tumors of the chest wall (all rib). Event-free survival at 5 years was 57% for the chest wall compared with 61% for other sites (P >.2). Ifosfamide and etoposide improved outcome in the overall group (5-year event-free survival, 68% vs 54%; P =.002), and a similar trend occurred in chest wall lesions (5-year event-free survival, 64% vs 51%). Patients with chest wall lesions had more attempts at initial surgical resection (30%) than those with other primary tumor sites (8%, P <.01). The attempt at initial resection for chest wall lesions did not correlate with size. Initial resections at other sites were restricted to smaller tumors. Initial resection resulted in negative pathologic margins in 6 of 16 patients, whereas the delayed resection resulted in negative margins in 17 of 24 patients (P =.05). Although there was no difference in survival by timing of the operation in rib lesions, a higher percentage of patients with initial surgical resection received radiation than those with resection after initial chemotherapy (P =. 13). CONCLUSIONS: Although rib primary tumors are significantly larger than tumors found in other sites, their outcome is similar. We favor delayed resection whenever possible to minimize the number of patients requiring radiation therapy.

Adolescent↗
PubMed2004

Tumor cells are present in stem cell harvests of Ewings sarcoma patients and their persistence following transplantation is associated with relapse.

BACKGROUND: Tumor cells frequently contaminate autologous stem cell products in a variety of malignancies, but their clinical significance remains controversial. We retrospectively monitored tumor contamination in stem cell harvests from patients with Ewing family of tumors (EFT) all harboring the specific translocation EWS-FLI-1 that characterize these tumors. PROCEDURE: Twenty- seven harvests from 11 patients were included in the study. In addition, 6 and 19 bone marrow (BM) or peripheral blood (PBL) samples were available before and after transplantation, respectively, for RT-PCR and nested PCR analyzes. RESULTS: All 11 patients had contaminating tumor cells in their harvests. All samples prior to transplantation were RT-PCR positive. Two out of the 11 patients who underwent transplantation died of complications. Out of the remaining nine patients, two are alive and well 68 and 84 months from diagnosis, and are the only patients with no detectable tumor cells in their samples after transplantation. One of these patients harbored contaminating tumor cells in only one of the two harvests collected. Seven patients relapsed after transplant, and in four patients BM/PBL samples were available prior to the clinical relapse. All these samples harbored contaminating tumor cells. CONCLUSIONS: We suggest a possible correlation between the amount of contaminating cells in the harvest and relapse after transplantation. Quantitative RT-PCR studies of the chimeric transcripts are underway to explore this issue.

Adolescent↗
PubMed2004

Ewing sarcoma/peripheral primitive neuroectodermal tumor: adult abdominal tumors with an Ewing sarcoma gene rearrangement demonstrated by fluorescence in situ hybridization in paraffin sections.

The differential diagnosis of small round cell tumors is exhaustive and requires ancillary studies. Relatively recently, fluorescence in situ hybridization (FISH) using probes for specific gene rearrangements has gained wide acceptance. This technique is particularly useful in the differential diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) and desmoplastic small round-cell tumor (DSRCT). In ES/PNET, the EWS gene is juxtaposed to the FLI-1 gene in 85% of cases and to the ERG gene in another 7% of cases; the EWS gene is juxtaposed to the WTI gene in DSRCT. Documentation of the EWS gene rearrangements in EWS/PNET has previously been demonstrated in frozen tissue. We report 2 unusual cases of EWS/PNET diagnosed in abdominal tumors in adults. Although the immunohistochemical results supported a diagnosis of ES/PNET, 1 case morphologically resembled DSRCT. The diagnosis in these 2 cases was confirmed by the FISH demonstration of EWS/FLI-1 gene fusion in paraffin-embedded tissue. Thus, the usefulness of FISH demonstration of an EWS gene rearrangement with these specific probes in such unusual cases is supported and is demonstrated in paraffin-embedded tissue.

Abdominal Neoplasms↗
PubMed1999

Extraskeletal Ewing's sarcoma in a kidney transplant patient.

Organ transplant recipients are prone to develop a variety of malignancies, most of which are encountered uncommonly in the general population. Approximately 5% to 7% of these malignancies are sarcomas, of which most are Kaposi's sarcomas. Ewing's sarcoma is an extremely uncommon tumor in organ transplant recipients, and only one case of skeletal Ewing's sarcoma has been reported in the transplant literature. We present a case of extraskeletal Ewing's sarcoma (EES) in a renal transplant patient.

Abdominal Muscles↗
PubMedDate not supplied

[Local therapy of Ewing sarcoma: radiotherapy aspects].

In CESS 86, radiotherapy and surgery as local treatment modalities yielded the same survival rates. Irradiated patients developed more local recurrences as compared to surgically treated patients (14% versus 4%), but less systemic metastases (16% versus 28%). The local recurrence rate after definitive radiotherapy dropped from 50% in CESS 81 to 14% in CESS 86. This was probably caused by an earlier start of radiotherapy (in week 10 in CESS 86 as compared to week 19 in CESS 81) and the high quality of radiation therapy in CESS 86 due to central treatment planning (only 2% protocol violations). Patients with local recurrences showed no differences in the distribution of major prognostic parameters (tumor volume, response to chemotherapy) as compared to patients with local control of disease. As a consequence of these results the concept of early irradiation with subsequent "consolidant" surgery for high-risk patients has been established.

Adolescent↗
PubMedDate not supplied

Brachytherapy in the combined modality treatment of pediatric malignancies. Principles and preliminary experience with treatment of soft tissue sarcoma (recurrence) and Ewing's sarcoma.

Radiotherapy is an integral part in the treatment of soft tissue and Ewing's sarcoma in children. By brachytherapy a high dose can be delivered in a restricted volume with sparing of normal tissues surrounding the target. Taking into account this principle potential benefit brachytherapy may play some role in the local treatment especially in children. However, only limited experience with pediatric brachytherapy has been reported apart from a few centers, which have gained their experience with Low-Dose-Rate (LDR)-brachytherapy. Since 1991 - 12 patients with soft tissue sarcoma and 6 patients with Ewing's sarcoma were treated with High-Dose-Rate (HDR) and Pulse-Dose-Rate (PDR)-brachytherapy at the departments of radiotherapy in Münster, Kiel and Vienna. The combined modality treatment was performed according to the CWS-86/91, EICESS-92 and CESS/CWS-REZ-91 protocols. In 8 patients with soft tissue sarcoma brachytherapy was part of the recurrence treatment regime, in 4 patients brachytherapy was part of the primary treatment alone or in combination with external beam therapy. In HDR-treatment a dose of 15 to 43 Gy was delivered in 3 to 16 fractions, in PDR-treatment 13 to 36 Gy in fractions of 1 Gy/hour. Follow-up is 3-39 months (median 14 months). 7 patients show no evidence of disease, 9 patients are locally controlled and 3 patients progressed locoregionally. In 6 patients with Ewing's sarcoma brachytherapy was performed intraoperatively as a boost treatment after external beam therapy (50-55 Gy), if no wide resection could be achieved within first line-treatment. A dose of 10-12 Gy was applied in one fraction in a limited volume (20-50 ccm) at the time of surgery. Follow-up is 13-26 months (median 21 months). There is no evidence of disease in all patients, perioperative and subacute morbidity was not increased. These encouraging preliminary results with HDR/PDR-brachytherapy must be further evaluated prospectively and systematically within an interdisciplinary approach by some specialized collaborating centers, which not only have the equipment (HDR/PDR/(LDR)-brachytherapy) but also can meet the complex demands to accumulate the necessary experience.

Adolescent↗
PubMedDate not supplied

[Cooperative Ewing's Sarcoma Studies 81/86: pathologico-anatomic and immunohistochemical findings and differential diagnosis of Ewing sarcoma].

158 cases of the Cooperative Ewing's Sarcoma Trials (CESS 81/86), which have been documented at the Pediatric Tumor Registry, Kiel, were studied by conventional light microscopy and immunohistochemistry. There were 77 cases of typical Ewing's sarcoma with 70 cases being located in the skeleton and 7 in soft tissues. Of the 14 cases of atypical Ewing's sarcoma 7 cases each were localized in bone and in soft tissue, respectively. In contrast to typical Ewing's sarcoma, cells of atypical Ewing's sarcoma were larger and displayed more heterochromatin. Both, typical and atypical Ewing's sarcoma reacted positively for vimentin. Other stains were negative, notably the neuron specific enolase (NSE). In 55 cases a diagnosis of malignant peripheral neuroectodermal tumor (MPNT) was made. Histologically most of these tumors resembled atypical Ewing's sarcoma. By immunohistochemistry positive reactions were found for NSE, vimentin, protein S-100, neurofilaments and glial fibrillary acidic protein. In 3 cases a diagnosis of small cell osteosarcoma was made. There were 2 cases of undifferentiated sarcoma of bone, 2 cases of soft tissue sarcoma of undetermined histogenesis and 2 cases of rhabdomyosarcoma. Of the 4 tumors which could be investigated for response to polychemotherapy, 1 each corresponded to grade II and III, respectively, and 2 to grade IV according to the classification of histologic grade of regression established by Salzer-Kuntschik et al. (1983).

Adolescent↗
PubMed2002

[Periosteal Ewing's sarcoma].

Periosteal Ewing's sarcoma is a histologically typical Ewing's sarcoma arising in the periosteum with no involvement of the medullary canal or cancellous bone. We describe four cases in our experience and review the literature, recalling the usual computed tomography diagnostic criteria and the therapeutic consequences. Prognosis of periosteal Ewing's sarcoma is generally better than for ordinary Ewing's sarcoma. In order to avoid inoculating the canal, the operator must strictly avoid perforating the cortical during the biopsy. At surgical resection, cortical resection can be partial maintaining a continuous diaphysis; this should be examined as a possibility for young patients in order to avoid the problems encountered with massive reconstruction followed by chemotherapy.

Adolescent↗
PubMed2005

Expression of the EWS/FLI-1 oncogene in murine primary bone-derived cells Results in EWS/FLI-1-dependent, ewing sarcoma-like tumors.

Ewing sarcoma is the second most common malignant pediatric bone tumor. Over 80% of Ewing sarcoma contain the oncogene EWS/FLI-1, which encodes the EWS/FLI-1 oncoprotein, a hybrid transcription factor comprised of NH2-terminal sequences from the RNA-binding protein EWS and the DNA-binding and COOH-terminal regions of the Ets transcription factor FLI-1. Although numerous genes are dysregulated by EWS/FLI-1, advances in Ewing sarcoma cancer biology have been hindered by the lack of an animal model because of EWS/FLI-1-mediated cytotoxicity. In this study, we have developed conditions for the isolation and propagation of murine primary bone-derived cells (mPBDC) that stably express EWS/FLI-1. Early-passage EWS/FLI-1 mPBDCs were immortalized in culture but inefficient at tumor induction, whereas later-passage cells formed sarcomatous tumors in immunocompetent syngeneic mice. Murine EWS/FLI-1 tumors contained morphologically primitive cells that lacked definitive lineage markers. Molecular characterization of murine EWS/FLI-1 tumors revealed that some but not all had acquired a novel, clonal in-frame p53 mutation associated with a constitutive loss of p21 expression. Despite indications that secondary events facilitated EWS/FLI-1 mPBDC tumorigenesis, cells remained highly dependent on EWS/FLI-1 for efficient transformation in clonogenic assays. This Ewing sarcoma animal model will be a useful tool for dissecting the molecular pathogenesis of Ewing sarcoma and provides rationale for the broader use of organ-specific progenitor cell populations for the study of human sarcoma.

Amino Acid Sequence↗