Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1261473 (sarcoma)
 25,952 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Four cases of a locally invasive sarcoma of the chest wall are described in children aged between 8 and 14 years. Although its morphology resembles a Ewing's sarcoma of bone there are light and electron microscopical features that are distinctive. The sarcoma is thought to represent an entity which has not previously been defined. Its clinical importance lies in the fact that the prognosis with appropriate treatment appears to be good. One child died after 2 1/2 years, but the others are alive 6, 8, and 16 years after presentation.
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PMID:Chest wall sarcoma of childhood with a good prognosis. 72 12

Between August 1984 and January 1987, the Southwest Oncology Group (SWOG) registered 46 patients with metastatic sarcomas on SWOG 8465, a Phase II trial of high-dose cisplatin in patients with metastatic soft tissue sarcoma. Six patients were ineligible for the following reasons: poor performance status (two patients); ineligible diagnosis (three patients, two with Ewing's sarcoma of bone and one with metastatic chondrosarcoma); and evaluable but nonmeasurable disease (one patient with bone-only disease). Of the 40 fully evaluable patients, 34 had received prior chemotherapy; treatment was with cisplatin (40 mg/m2/d for 5 consecutive days). Cisplatin was mixed in 250 ml of 3% NaCl and hydrated with a normal saline solution at a rate of 250 ml/h, beginning 12 hours before the first dose of cisplatin was specified. The second treatment was given 3 weeks after the first, with all subsequent treatments given every 4 weeks. After three cycles of treatment, responding patients were treated at a cisplatin dose of 20 mg/m2/d for 5 consecutive days. Leukopenia was of Grade 3 or 4 in seven patients, whereas thrombopenia was of Grade 3 or 4 in eight patients. More severe myelosuppression was produced in patients who had received prior radiotherapy. A single case of reversible Grade 4 nephrotoxicity was produced; neurotoxicity was observed in 11 cases, but was of Grade 3 in only 2 cases. Of the 40 evaluable cases, six showed partial responses or no responses, for a major response rate of 15%. High-dose cisplatin has minor activity and major toxicity in the treatment of metastatic soft tissue sarcomas, and should be considered investigational.
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PMID:High-dose cisplatin for metastatic soft tissue sarcoma. 229 55

Ewing's sarcoma is a distinctive clinical and pathologic entity that may be intraosseous or of soft-tissue origin. In the facial bones, the incidence of the sarcoma is estimated at 2.5% of all Ewing's sarcoma of bone. The soft tissue of the head and neck account for 11% of extraskeletal sites of the neoplasm. Combined modality treatment has modified its high-grade malignancy.
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PMID:Ewing's sarcoma of bone and soft tissues. 265 27

Unlike the osseous variety, extraskeletal small cell tumors histologically identical to Ewing's sarcoma of bone frequently respond to radical surgical excision. The relative infrequence of this tumor, compounded by the comprehensive eponym "Ewing's sarcoma," may deter surgical intervention in this group of patients. This patient's course demonstrates the value of surgery alone, but emphasizes the role of combined surgery, irradiation, and chemotherapy as the best management for extraskeletal Ewing's sarcoma.
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PMID:Extraskeletal Ewing's sarcoma: prolonged survival with recurrence after operation. 741 94

Extraosseous Ewing's sarcoma is an undifferentiated round-cell sarcoma that is indistinguishable histologically from Ewing's sarcoma of bone. Despite this similarity, it has been accepted as a distinct clinicopathologic entity. The authors describe a 53-year-old man who presented with extraosseous Ewing's sarcoma of the left subcostal chest wall. The lesion was demonstrated by radiography and computed tomography and confirmed by pathological examination.
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PMID:Extraosseous Ewing's sarcoma. 770 78

High-dose chemoradiotherapy (HDCRT) followed by autologous stem cell (ASC) rescue is now widely used in a number of childhood malignancies. The most common to date is neuroblastoma, where it now has an established role as consolidation of initial complete remission in children over the age of 1 year with stage 4 disease. High-dose melphalan alone prolongs progression-free survival, with a small increase in long-term survival. The value of a total body irradiation (TBI)-based regimen is currently under randomized evaluation. In soft-tissue sarcoma, such as rhabdomyosarcoma or Ewing's sarcoma of bone or soft tissue, high-dose therapy has been used to consolidate initial complete or partial remission. The benefit has not been demonstrated in randomized studies but chemotherapy-alone regimens based on combined alkylating agents appear to be of potential value. Other tumours have been treated in this way but the place of high-dose therapy remains entirely unclear. It is possible that inherently chemosensitive tumours, such as Wilm's tumour, may be candidates where standard treatment has failed. The replacement of bone marrow reinfusion with cytokine-primed peripheral blood stem cell (PBSC) rescue has reduced the morbidity of these procedures. The issue of the potential risk of reinfusing tumour cells has been addressed in neuroblastoma where purging using immunodepletion or in vitro chemotherapy has been carried out.
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PMID:Autologous stem cell transplantation in solid tumours of childhood. 873 45

A report on the long-term follow up of the first cooperative Scandinavian Sarcoma Group study in Ewing's sarcoma of bone is presented. Fifty-two previously untreated patients entered the study between 1984 and 1990. Half of the tumors were located in the extremities and one quarter in the pelvis. The combined modality treatment consisted of 5 cycles of chemotherapy--including vincristine, methotrexate, doxorubicin, cyclophosphamide, bleomycin and dactinomycin. The first two cycles were followed by local resection or amputation in 35 patients and by radiotherapy alone in 17 patients. When surgery was not performed, was incomplete or yielded poor margins radiotherapy was given at a dose of 40-60 Gy. Local tumor relapses developed in 10 patients and in all but one patient were accompanied by metastatic disease. Five patients had metastasis at diagnosis and distant metastases developed after primary treatment in 27 patients after a median of 14 months. The median follow-up time for the 20 surviving patients is 10 years. At 5 years the tumor-related survival was 46% and the metastasis-free survival 43%. Late tumor relapses occurred in 4 patients, which reduced the 10-year tumor related survival to 41% and the metastasis-free survival to 38%. Histopathological tumour response correlated with survival with 5-year metastasis-free survival rates of 73% for the good responders and 35% for the poor responders.
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PMID:Ewing's sarcoma treatment in Scandinavia 1984-1990--ten-year results of the Scandinavian Sarcoma Group Protocol SSGIV. 974 60

All cases of high-grade osteosarcoma (OS) (n = 196) and Ewing's sarcoma of bone (ES) (n = 56) treated at the Norwegian Radium Hospital in the period 1980-1999 were analyzed retrospectively. They were allocated to consecutive ten-year periods by their time of diagnosis. Patient and tumour characteristics have been relatively stable. Eighty percent of all patients received surgical treatment and the amputation rate decreased from 64% to 23%. The percentage of patients receiving chemotherapy has remained around 80%. The use of radiotherapy in primary treatment decreased gradually from 33% to 18%. Sarcoma specific survival (SSS) at five years for all patients increased significantly from 39% to 53%. Similar trends for improvement were seen for both OS and ES. In multivariate analysis, independent prognostic factors for improved SSS were non-metastatic disease at diagnosis, age under 40, extremity tumours, small tumours and treatment from 1995 onwards. No major new treatment options have emerged over these 20 years. The improved outcome appears partly to be due to refinements in the use of existing modalities and improved quality and integration of multidisciplinary approaches. Improved formalized organisation of the sarcoma group and annual audited reports of its patient and research activity may also have contributed to improved focus and performance.
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PMID:Management of high-grade bone sarcomas over two decades: the Norwegian Radium Hospital experience. 1646 94

Purpose. Improvements in the systemic management of Ewing's sarcoma of bone over the last 20 years have led to a dramatic improvement in survival. The corollary is that treatment of the primary disease requires re-evaluation, since a significant number of patients still suffer local relapse.Patients. The effect of radiation dose on local control was reviewed in a series of 96 patients treated between 1967 and 1986. Seventy-four had no metastases at presentation (M0), 22 had metastases (M1). The 5-year survival of all patients was 28%, and of M0 patients alone 37%. Although these figures are poor by today's standards, they are consistent with published studies whose patients were enrolled during the same calendar period. Although most deaths occurred by 5 years, survival continued to fall beyond 10 years, which has implications for follow-up in future studies.Results. The local control (LC) rate at 5 years was 56% for all patients and for M0 patients analyzed separately. There was no difference in either LC or survival between the first and second decades of the study. Primary site was a significant determinant of survival and local control, with better outcome for limb tumours compared to pelvic primaries. Chemotherapy also had a major effect on LC. Radiotherapy improved the probability of LC. Omission of radiotherapy, or a dose <40 Gy, was ineffective. In the dose range 40-66 Gy, there was no evidence of a dose-response relationship.
Sarcoma 1997
PMID:The Impact of Radiotherapy Dose on Local Control of Ewing's Sarcoma of Bone. 1852 Nov 98

Extraskeletal Ewing's sarcoma is a rare soft tissue tumor that is morphologically indistinguishable from Ewing's sarcoma of bone. We report a case of extraskeletal Ewing's sarcoma with several systemic problems. A 69-year-old man presented with a 5-month history of a rapidly enlarging mass in the right thigh. Because preoperative radiotherapy with sanazole (AK-2123) contributed the tumor mass reduction down to 40% in size, the tumor was successfully resected with clear surgical margins and repaired with a musculocutaneous flap. The high efficacy of pre-operative low-dose radiotherapy with sanazole was histologically confirmed that the resected tumor specimen involved no viable tumor cells and showed 100% necrosis. Based on clinical outcomes in this case, the combined modality of pre-operative low-dose radiotherapy with hypoxic cell radiosensitizer and adequate surgical resection might provide for the useful clinical application of extraskeletal Ewing's sarcoma treatment.
Sarcoma 2011
PMID:High Efficacy of Preoperative Low-Dose Radiotherapy with Sanazole (AK-2123) for Extraskeletal Ewing's Sarcoma: A Case Report. 2087 55


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