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Small round blue cell tumors in bone: prognostic factors correlated to Ewing's sarcoma and neuroectodermal tumors.
Ewing's sarcoma and peripheral neuroectodermal tumors are the most common small round blue cell tumors of bone. Accurate prognostic factors are required to define guidelines to standardize the treatment modalities and to adapt these modalities to the potential evolution of the disease. The various factors that have emerged in the literature as influences on the outcome of patients with Ewing's sarcoma or peripheral neuroectodermal tumors of bone are considered in this review. The presence of metastases at the clinical onset of the disease represents the most adverse prognostic factor. For nonmetastatic patients, axial location appears to be the most unfavorable factor, despite initial tumor volume, by showing that the tumor burden could be a more appropriate indicator of patient outcome. The importance of a local control of the disease by surgery has been emphasized, and the value of the histopathologic evaluation of the response to chemotherapy has been stressed.
Ewing's sarcoma of the nose.
Ewing's sarcoma is a bone tumor that rarely arises in the head and neck region. Head and neck tumors account for only 2% to 3% of all Ewing's sarcomas. There is an extremely rare subgroup of Ewing's tumors, that are histologically indistinguishable, which are not metastatic and arise in soft tissue rather than bone. Two Ewing's sarcomas of the nose are described. One case is the first reported nasal Ewing's soft tissue sarcoma. The other case is the more traditional type involving bone of the nose and extending into ethmoid sinuses and intracranially.
[Chromosomal translocation (11; 22) in cell lines of Ewing's sarcoma].
Chromosome studies were performed on 5 Ewing sarcoma cell lines. An identical reciprocal translocation t(11; 22) (q24; q12) was found in 4 cell lines established from 3 different tumors. These results, associated with those obtained at the same time and independently from fresh tumor cells, suggest that the translocation t(11; 22)(q24; q12) may be a chromosomal marker characteristic of Ewing sarcoma cells. This translocation involves the chromosome 22 on which the H-c-sis oncogene has been located; it could be used as a new tool for exploring the role of genetic transposition in the malignant cell transformation.
Past failures and future possibilities in Ewing's sarcoma. Experimental and preliminary clinical results.
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Novel oncogenic mutations in the WT1 Wilms' tumor suppressor gene: a t(11;22) fuses the Ewing's sarcoma gene, EWS1, to WT1 in desmoplastic small round cell tumor.
These studies suggest that the WT1 tumor suppressor gene, originally identified as a recessive oncogene in Wilms' tumors, is capable of sustaining a gain-of-function mutation which results in its contribution to a completely different disease entity: desmoplastic small round cell tumor. Two independent biochemical functions of WT1, DNA-binding activity and mode of transcriptional regulation, are altered as a consequence of the chromosomal translocation and fusion with EWS. The fusion of EWS and WT1 genes in DSRCT thus provides a unique paradigm for a means by which different alterations of transcription factor function can lead to diverse oncogenic processes.
Establishment and characterization of a clonal human extraskeletal Ewing's sarcoma cell line, EES1.
Ewing's sarcoma, a small round cell sarcoma arising in soft tissue as well as the bone, is one of the most malignant tumors in children and young adults. Few established cell lines of extraskeletal Ewing's sarcoma (EES) have been reported, which made it difficult to examine the biological features of EES. Therefore, we have established a new clonal cell line of EES. We report its morphological characters, results of chromosomal and immunohistochemical analysis. A piece of tumor obtained from the 18-year-old female patient with EES was xenografted in a nude mouse. In vitro subcultured cells were then obtained from this xenograft. A clonal cell line was subsequently established by limiting dilution and designated EES1. EES1 cells had a doubling time of 24 hours. In the xenografted tumor, the cells expressed vimentin, CD99 (MIC2), neuron specific enolase (NSE) and cytokeratin. The original tumor cells also expressed vimentin, CD 99, and NSE, but was negative for cytokeratin. The morphological and immunohistochemical features of this cell line established, except for cytokeratin expression, were consistent with those of the primary tumor. Cytogenetic analysis of EES1 revealed chromosomal translocation of t(11; 12)(q24;ql2). The chimeric fusion of the Ewing's sarcoma gene in band 22q12 with the Friend leukemia virus integration-1 gene in band 11q24 was also demonstrated. Fluorescence in situ hybridization further confirmed the presence of translocation involving the Ewing's sarcoma gene in both the primary tumor and EES1 cells. In conclusion, we have established a human EES cell line EES1, which will provide a useful model for studying various aspects of human EES.
Ewing's sarcoma masquerading as osteomyelitis.
Ewing's sarcoma in the infant and young child is rare, highly malignant, and can be difficult to identify. An erroneous diagnosis of osteomyelitis may be considered first because the presentation may be similar to that of Ewing's sarcoma, and routine laboratory evaluation may not distinguish between these entities. Two such cases are presented, one involving the tibia in a 16-month-old child and another a finger phalanx in a 7-month-old child. In both cases the correct diagnosis of Ewing's sarcoma was delayed because of initial misdiagnosis of osteomyelitis. This diagnostic dilemma is summarized, and the literature reviewed. Special attention is given to recent advances in histochemistry and cytogenetics that assist in tumor identification. The conclusion highlights areas of remaining controversies for which additional study may facilitate distinction between osteomyelitis and Ewing's sarcoma.
Functional results after partial pelvic resection in Ewing's sarcoma of the ilium.
Ewing's sarcoma of the pelvis has an unfavourable prognosis. The clinical and functional results of 7 patients who had a Ewing's sarcoma of the pelvis stage IIB were reviewed. All patients received multiple-agent chemotherapy pre- and postoperatively (modified T6 and T2 protocol according to Rosen) and underwent local resection of the pelvic tumour. According to Enneking, five patients had a type IA resection, one patient type I and another type IIA. One patient received a course of radiation therapy postoperatively (50 Gy). Six of seven patients showed a good regression of the tumour after preoperative chemotherapy. One patient who had a giant-cell Ewing's sarcoma died of local recurrence and lung metastases 29 months postoperatively. The remaining six patients were monitored radiographically and clinically according to Enneking's functional evaluation score after a follow-up period of 136 months (range 40-199 months). All were free of disease and had neither local recurrence nor metastases. In five patients the functional results were rated as "good" or "excellent". The good results depend mainly on the reconstruction of the pelvic girdle and its mechanical stability.
Acute nonlymphocytic leukemia developing during the course of Ewing's sarcoma.
Two children with Ewing's sarcoma developed acute nonlymphocytic leukemia (ANLL) during the course of their illness. One patient developed ANLL after apparently successful treatment of his primary malignancy with radiation therapy and multiagent chemotherapy. In the second patient, acute leukemia developed before the administration of radiotherapy or systemic chemotherapy. The development of secondary ANLL after Ewing's sarcoma has been reported only twice previously, most likely representing a therapy-induced complication. The occurrence of ANLL in Patient 2 prior to therapy suggests that these two disorders may have a more than treatment-related association. Close follow-up of long-term survivors of Ewing's sarcoma with surveillance for secondary acute leukemia is advised.
Ewing's sarcoma of the mandible.
Ewing's sarcoma of the head and neck, particularly of the mandible, has a low incidence of occurrence (fifty-five cases in the literature). The present case report demonstrates well the clinical, diagnostic, and therapeutic features in a patient treated at the Head and Neck Service of the Fundação Antonio Prudente, São Paulo, Brazil. The etiologic, clinical, histopathologic, and radiologic features are discussed, with the conclusion that radiation is the best therapeutic procedure when associated with chemotherapy.
Present trends in treatment of Ewing's sarcoma.
The prognosis of Ewing's sarcoma, the first malignant tumor in children under 10, has improved dramatically in the last 10 yrs. The authors review the signs and symptoms that permit diagnosis and analyse the improvement in treatment, based on multidrug's combination, neoadjuvant chemotherapy and surgical treatment of the primary. They point out the necessity of closed multidisciplinary co-operation to treat these patients. Under these conditions, disease free survival now reaches more than 90% at 3 years 1/2.
Treatment strategies for metastatic Ewing's sarcoma.
Therapy in metastatic Ewing's sarcoma is reviewed using the methodology recommended by the guidelines project of the Federation of French Cancer Centres (FNCLCC) Standards, Options and Recommendation (SOR) Group. Twelve articles relating to conventional dose therapy and seven articles related to high-dose therapy were judged suitable for detailed appraisal. Rates of complete response (CR) at metastatic sites and local control were high using combinations of vincristine, actinomycin, cyclophosphamide and doxorubicin with radiation or surgery. With more recent regimens, including increased doses of alkylating agents and anthracyclines the relapse-free survival has increased from <15 to 20-30%. 'Megatherapy' regimens with haematopoietic stem cell rescue are tolerable in this patient group, but to date there is little evidence of any benefit. It appears that patients with isolated lung metastases do significantly better (approximately 40% EFS) than those presenting with combined sites such as bone, bone marrow and lung. The use of lung irradiation in children with lung metastases is associated with a reduced incidence of subsequent lung recurrence and a consistently better overall relapse-free survival (RFS).
Radiologic identification of Ewing's sarcoma.
The authors discuss Ewing's sarcoma, a rare pedal neoplasm. They concentrate on radiologic interpretation of this entity. Case studies are illustrated.
Ewing's sarcoma of the retropharynx.
Ewing's sarcoma arising in the head and neck region is very rare. A case arising from C2 in a young child is presented. The unusual presentation, differential diagnosis and pathological features are discussed.
Ewing's sarcoma of the mandible.
Ewing's sarcoma of the mandible occurs rarely; there have been less than 50 reported cases in the past 30 years and none of these have been in the otolaryngologic literature. They usually present in the second decade of life with pain and swelling of the soft tissue overlying the mandible. The diagnosis can only be made by an adequate mandibular biopsy, although x-rays and clinical presentation are helpful. Current treatment consists of radiation therapy and sequential adjuvant chemotherapy.