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Ewing's sarcoma of the mandible.

Ewing's sarcoma is an uncommon neoplasm that primarily occurs in the long bones of the lower and upper extremities. Only 2% involve the mandible, and the maxilla is involved one eighth as often as the mandible. The most common site of metastases is the lung. Increasing pain is the most common presenting symptom. A case of mandibular involvement is presented. The combination of a large, soft tissue mass adjacent to an area of bone destruction in a young male should suggest the diagnosis. The previously poor five- and ten-year survival rates of 8 and 4% respectively, may have promise of marked improvement. This improvement lies in a changing philosophy of treatment. Four-drug chemotherapy combined with prophylactic whole-lung irradiation has been added to local irradiation of the tumor bed. Five-year survival figures of 20 to 30% appear obtainable. Although extensive surgical resection of the primary tumor in the long bones has for the most part been replaced by local irradiation for control, surgery in mandibular cases may still be the treatment of choice.

Child↗
PubMed1987

Function after partial pelvic resection for Ewing's sarcoma.

Seven patients with Ewing's sarcoma of the pelvis were treated by chemotherapy followed by wide resection of the primary tumour. Although good function after operation is possible, survival in this series reflects the poor prognosis associated with the disease; two patients died, two are alive with local recurrence and metastases and three patients are alive with no evidence of disease.

Adolescent↗
PubMed1999

Phosphoinositide 3-hydroxide kinase blockade enhances apoptosis in the Ewing's sarcoma family of tumors.

Ewing's sarcoma family of tumors (ESFTs) affects patients between the ages of 3 and 40 years, with most cases occurring in the second decade of life. These tumors contain a characteristic translocation, t(11;22), that produces a unique fusion protein, EWS/FLI-1. EWS/FLI-1 transforms mouse fibroblasts; this transformation requires intact EWS and FLI-1 domains as well as the insulin-like growth factor-I receptor (IGF-IR). The IGF-IR is a well-described transmembrane tyrosine kinase receptor that modulates transformation, cell growth, and survival. IGF-IR survival signaling is mediated through the downstream activation of phosphoinositide 3-OH kinase (PI 3-K) and Akt. Apoptosis, programmed cell death, progresses from a central death signal to a caspase cascade, including activation of caspase-3. Because the IGF-IR has been shown to play a role in the transformation and growth of ESFTs, we wanted to determine the role of downstream molecules in the cellular response to doxorubicin treatment. Doxorubicin increased caspase-3 activity in two ESFT cell lines, TC-32 and TC-71. Concomitant treatment of the doxorubicin-treated cells with IGF-I reduced caspase-3 activity 8-fold in TC-32 and 4-fold in TC-71. To determine whether PI 3-K has a role in IGF-I-mediated survival in ESFTs, PI 3-K was then inhibited with wortmannin and LY294002. Doxorubicin treatment reduced cell number and enhanced apoptosis in PI 3-K inhibited cells compared with noninhibited cells. Akt, a serine/threonine kinase activated downstream of PI 3-K, was investigated to determine whether its constitutive activation would render ESFT cells more resistant to doxorubicin. A constitutively activated Akt was stably transfected into ESFT and those cells with high levels of expression demonstrated increased resistance to doxorubicin-induced caspase-3 activation. These results indicate that ESFT cell lines use an IGF-IR initiated signaling pathway through PI 3-K and Akt for survival when treated with doxorubicin.

Androstadienes↗
PubMed1994

Immunohistochemical profile of monoclonal antibody O13: antibody that recognizes glycoprotein p30/32MIC2 and is useful in diagnosing Ewing's sarcoma and peripheral neuroepithelioma.

Ewing's sarcoma (ES) and peripheral neuroepithelioma (PN) are closely related tumors, and it can be difficult to distinguish them from other small-round-cell tumors (SRCTs). The glycoprotein p30/32MIC2 is highly, but not exclusively, expressed in both ES and PN. Although the monoclonal antibody (Mab) HBA71, which reacts with P30/32MIC2, has been reported to be relatively specific and highly sensitive for both neoplasms, it is not readily available. Yet, Mab O13 is commercially available, and it purportedly displays the same immunostaining characteristics as HBA71. Because O13 has not been studied extensively, we immunostained 21 ES/PNs and 147 other tumors or lesions that might show SRCT-like features with O13. The results were similar to those reported for HBA71. We found O13 to be 100% sensitive for ES/PN; and, no immunostaining was noted on the SRCTs often included in the differential diagnosis of ES/PN (i.e., conventional neuroblastoma, rhabdomyosarcoma, and non-lymphoblastic lymphomas). But, O13 immunoreacted with lymphoblastic lymphomas and some other tumors and normal tissues. Nonetheless, this nonspecific reactivity should not cause diagnostic problems, if an antibody panel containing anti-desmin and anti-leukocyte common antigen is used in conjunction with O13. We conclude that, within the proper diagnostic context, strong immunoreactivity of a SRCT tumor for O13 should be considered good evidence that the tumor is ES/PN.

12E7 Antigen↗
PubMed1989

Ewing's sarcoma of the foot.

Ewing's sarcoma is a rare, malignant neoplasm that is most common in children and adolescents. It is common in both the axial and appendicular skeleton but has been rarely reported in the foot. Historically, treatment has consisted of chemotherapy and radiation therapy. The roll of surgery in the management of these lesions have recently been "rediscovered" and depends on the anatomic location. The purpose of this paper is to discuss five consecutive cases of Ewing's Sarcoma of the forefoot treated with below-the-knee amputation for local tumor control. Four of the five patients also underwent some systemic chemotherapy. After comparing the various forms of therapy for this tumor, the benefits of below-the-knee amputation make it an excellent primary treatment for Ewing's sarcoma of the foot.

Adolescent↗
PubMed1976

Prophylactic irradiation of the lung in Ewing's sarcoma.

A patient with Ewing's sarcoma of the left scapula received prophylactic radiotherapy to the left lung following wide excision of the tumor. While the left lung remained free of tumor, there was metastatic invasion of the right lung within ten months of the initial diagnosis of the disease. Since Ewing's sarcoma frequently metastasizes to the lungs, it is suggested that prophylactic irradiation of the lung may be of value in the prevention of metastases.

Adult↗
PubMed1987

Isochromosome 11q in a case of Ewing's sarcoma.

A low passage Ewing's sarcoma cell line established from a metastatic lesion was cytogenetically analyzed. The modal karyotype was 44,X, -8,i(11q), -15, +12. Other cells had random chromosome aneuploidy superimposed on this karyotype. No cell had a structural rearrangement involving 11q24 or 22q12 as described in other patients with Ewing's sarcoma, but all cells had an isochromosome 11q.

Child↗
PubMed1987

Ewing's sarcoma.

This patient had Ewing's sarcoma of the femur, which was originally misinterpreted by radiograph and by biopsy as osteomyelitis. For approximately 1 year the patient was treated for osteomyelitis. The lesion was slowly progressive and near the end of the first year became more active, and pulmonary metastases appeared. The natural behavior of this one case of Ewing's sarcoma demonstrates that it is not necessarily an overwhelmingly aggressive tumor. In addition, the point is made that pathologists should pay careful attention to necrotic material so that the suggestion of necrotic tumor can be made rather than dismissing the biopsy as nondiagnostic.

Biopsy↗
PubMed1983

Ewing's sarcoma of the jaws.

Ewing's sarcoma is an intraosseous malignant tumor of questionable pathogenesis which occurs only occasionally in the jaws. Seventeen cases, eight maxillary and nine mandibular, from the files of the Armed Forces Institute of Pathology (AFIP) are reported. This neoplasm primarily affects children and young adults. Swelling and pain are frequent symptoms. Radiographically, it is most often seen as a destructive, expansile, mottled radiolucent lesion, which may produce a laminated periosteal reaction. The prognosis is poor, although some reports of combined surgery, radiation, and chemotherapy protocols have offered encouragement for increased survival. Histopathologically, Ewing's sarcoma must be differentiated from other small, round-cell malignant neoplasms, such as neuroblastoma and reticulum cell sarcoma.

Adolescent↗
PubMed1992

[A clinical study of the continuous intra-arterial infusion chemotherapy of Ewing's sarcoma].

Eight cases of Ewing's sarcoma were treated by continuous intra-arterial infusion chemotherapy (i.a. infusion). Five cases gave no evidence of disease (mean 4 yr. 7 mos.), and 3 cases died (3 mos, 1 yr. 11 mo, 2 yrs. 5 mos.). The term of i.a. infusion was 3 mos. to 2 yrs. 3 mos. (mean 12 mos). Except in 2 cases, the primary tumors were suppressed by i.a. infusion, so these cases were not operated and no relapse resulted. The cumulative survival rate by Kaplan-Meier's method was 56.3%. The i.a. infusion for Ewing's sarcomas implies the possibility of limb-saving treatment with no operation.

Adolescent↗
PubMed1999

Adamantinoma-like Ewing's sarcoma: genomic confirmation, phenotypic drift.

Ewing's sarcoma, a highly malignant neoplasm, is characterized by an 11;22 translocation [t(11;22) (q24;q12)], resulting in the fusion of genes FLII and EWS. Adamantinoma of extragnathic bones, a low-grade malignant neoplasm with epithelial features, is not typically considered in the differential diagnosis of Ewing's sarcoma. In this study, three osseous Ewing's sarcomas with histological, immunohistochemical, or ultrastructural epithelial features were subjected to reverse transcription-polymerase chain reaction and sequencing studies for the Ewing's sarcoma molecular rearrangement. (Two of the three cases were originally described as adamantinomas or nontypical Ewing's sarcoma before the availability of genetic characterization.) In addition, traditional cytogenetic analysis and a unique combined interphase molecular cytogenetic/ immunocytochemical approach with bicolor 11;22 translocation breakpoint flanking probes (cosmids) and pancytokeratin antibodies were performed on one neoplasm. At(11;22) (q24;q12) was found in one neoplasm and a type II EWS/FLI-1 fusion transcript was detected in all three neoplasms. The combined genetic/immunocytochemical approach revealed the presence of the 11 ;22 translocation in the nuclei of cytokeratin immunoreactive cells. These genotypic and phenotypic findings delineate a novel Ewing's sarcoma histologic variant, "adamantinoma-like Ewing's sarcoma."

Adolescent↗
PubMed1979

Primary epidural Ewing's sarcoma presenting as a lumbar disc protrusion. Case report.

Primary neoplasms of the spinal epidural space are uncommon. One of the rarest of these is a soft tissue sarcoma indistinguishable from Ewing's sarcoma of the bone. Only 39 such cases have been reported previously, of which only three arose within the epidural space. The authors report an additional case, which presented in an atypical manner, and review the pertinent literature.

Adult↗
PubMed2000

Bone sarcomas of the head and neck in children: the St Jude Children's Research Hospital experience.

BACKGROUND: Bone sarcomas of the head and neck are difficult to resect. The authors reviewed their institutional experience with these tumors to characterize patients' clinical findings and to assess the impact of surgical resection on outcome. METHODS: The records of the 28 patients with bone sarcomas originating in the head and neck treated at St. Jude Children's Research Hospital between March 1962 and January 1998 were reviewed. RESULTS: There were 10 males and 18 females (median age, 12.6 years) each with a single sarcoma: osteosarcoma (18), Ewing sarcoma (7), malignant fibrous histiocytoma (MFH) (2), and fibrosarcoma (1). Primary tumor sites included the maxilla (13), skull (10), mandible (2), and other sites (3). All but one patient with Ewing sarcoma had localized disease at the time of diagnosis. All patients underwent surgery: complete resection, 8; gross total resection, 4; incomplete resection, 14; and biopsy only, 2; 22 also received chemotherapy. Radiotherapy was given to all patients with Ewing sarcoma and to four patients with primary osteosarcoma. Twelve patients survived a median of 8.4 years after diagnosis, 14 died of disease, and 2 died of unrelated causes. Local disease progression was evident in 12 patients (9 with osteosarcoma, 2 with MFH, and 1 with Ewing sarcoma) who died of disease, 9 of whom had the initial treatment of biopsy alone or incomplete resection. Patients with osteosarcoma who had the initial treatment of incomplete resection or biopsy alone were more likely to experience local failure (P = 0.001) and had poorer survival (P = 0.014) than those who underwent complete or gross total resection. CONCLUSIONS: Bone sarcomas of the head and neck are rare among children and most often are localized at the time of diagnosis. Incomplete resection of osteosarcoma is associated with local failure and poor outcome. Although aggressive surgery is essential for the cure of osteosarcoma, its necessity in the treatment of Ewing sarcomas remains controversial.

Adolescent↗
PubMed2006

Caveolin-1 (CAV1) is a target of EWS/FLI-1 and a key determinant of the oncogenic phenotype and tumorigenicity of Ewing's sarcoma cells.

Tumors of the Ewing's sarcoma family (ESFT), such as Ewing's sarcoma (EWS) and primitive neuroectodermal tumors (PNET), are highly aggressive malignancies predominantly affecting children and young adults. ESFT express chimeric transcription factors encoded by hybrid genes fusing the EWS gene with several ETS genes, most commonly FLI-1. EWS/FLI-1 proteins are responsible for the malignant phenotype of ESFT, but only few of their transcriptional targets are known. Using antisense and short hairpin RNA-mediated gene expression knockdown, array analyses, chromatin immunoprecipitation methods, and reexpression studies, we show that caveolin-1 (CAV1) is a new direct target of EWS/FLI-1 that is overexpressed in ESFT cell lines and tumor specimens and is necessary for ESFT tumorigenesis. CAV1 knockdown led to up-regulation of Snail and the concomitant loss of E-cadherin expression. Consistently, loss of CAV1 expression inhibited the anchorage-independent growth of EWS cells and markedly reduced the growth of EWS cell-derived tumors in nude mice xenografts, indicating that CAV1 promotes the malignant phenotype in EWS carcinogenesis. Reexpression of CAV1 or E-cadherin in CAV1 knockdown EWS cells rescued the oncogenic phenotype of the original EWS cells, showing that the CAV1/Snail/E-cadherin pathway plays a central role in the expression of the oncogenic transformation functions of EWS/FLI-1. Overall, these data identify CAV1 as a key determinant of the tumorigenicity of ESFT and imply that targeting CAV1 may allow the development of new molecular therapeutic strategies for ESFT patients.

Animals↗
PubMed2002

Cutaneous metastases from Ewing's sarcoma: report of two cases.

Ewing's sarcoma is a malignant osseous neoplasm that affects mostly children and young adult males. Clinically, the neoplasm presents with oedema, swelling, and pain of the involved area. Histopathologically, Ewing's sarcoma consists of solid sheets of small round cells, with vesicular nuclei and scant cytoplasm, arranged in irregular masses separated by strands of fibrous tissue, with areas of necrosis en masse intermingled with intratumoural haemorrhage. Ewing's sarcoma is an extremely aggressive neoplasm and metastases to sites such as lung, pleura, other bones, central nervous system, liver, and regional lymph nodes frequently develop in early stages of the disease. Surprisingly, despite the highly aggressive biological behaviour of this neoplasm, cutaneous metastases from Ewing's sarcoma are very uncommon. We report two patients with Ewing's sarcoma of the bone who developed cutaneous metastases. As in other internal malignancies, the onset of cutaneous metastases in patients with Ewing's sarcoma indicates a poor prognosis.

Adult↗
PubMed2001

[Primary Ewing's sarcoma of the cranial vault. Report of 2 cases].

Primary Ewing sarcoma of the calvarial skull is very rare, found in less than 1% of the cases. Frontal and parietal convexities are the common sites of occurrence. We report two cases of primary Ewing sarcoma of the skull (in 13- and 14-year-old boys. The first tumor involved the right temporal region and exhibited unclear osteolytic appearance on skull x-rays while computed tomography showed an extraaxial enhanced mass and bone sclerosis with spiculated periosteal reaction. In the second case, the tumor was temporoparietal. Plain films demonstrated a large osteolysis and computed tomography revealed extensive bone destruction involving both the inner and outer tables. In both cases, the C scan revealed extraaxial enhanced masses with intracranial and extracranial extension. Radial resection was performed. Adjuvant chemotherapy was given and no recurrence or metastasis has occurred two years later.

Adolescent↗
PubMed1991

Atypical primitive neuroectodermal tumors. Comparative light and electron microscopic and immunohistochemical studies on peripheral neuroepitheliomas and Ewing's sarcomas.

Recently, primitive neuroectodermal tumors (PNETs) have been shown to cover a wide spectrum of small round cell sarcomas, probably including some Ewing's sarcomas (ESs) and extraskeletal Ewing's sarcomas (EESs), in addition to classical peripheral neuroepitheliomas (PNs). In studies of small cell sarcomas, we found a group of undifferentiated tumors resembling PNETs with some features of neuroectodermal differentiation, but possessing areas of relatively large, pleomorphic cells. To clarify the nature of these tumors and their relationship to PNETs, we examined the variety of histological, immunohistochemical and ultrastructural features of 11 small cell sarcomas. Five of these tumors were composed of uniform, small round cells and were classified as PNs because of the presence of definite Homer-Wright rosettes and fibrillary processes. The presence of well developed neurite-like processes containing neurosecretory granules and immunoreactivities for various neural markers suggested that these PNs showed more advanced neuronal differentiation. Two tumors, with the classical features of ES, showed no ultrastructural evidence of neuronal differentiation, although only gamma-gamma neuron-specific enolase (NSE) positivity was detected. Four undifferentiated tumors with atypical features, included in this study as an atypical PNET group, showed certain neuroectodermal characteristics, such as ganglion cell differentiation, perivascular pseudorosettes, and gamma-gamma NSE reactivity. It is concluded from this study that PNETs may include small round cell tumors showing different degrees of neuro-ectodermal differentiation and some histological variations.

Adolescent↗