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1.
Purpose. The purpose of this study was to evaluate: (1) the correlation between grade and ploidy or S-phase fraction (SPF), (2) the prognostic value of DNA flow cytometric study in soft tissue sarcomas.Patients /Methods. In all, 47 tissue samples from soft tissue sarcoma patients, surgically treated in the same center, were included. Flow cytometric analyses were performed according to a modified version of the original method of Hedley et al.Results. DNA ploidy status could be determined in 44 samples out of 47 (success rate 94%). Of these 44, S-phase fraction could be calculated in 34 samples (77%). In the study group as a whole, aneuploidy was significantly correlated with high grade. Survival analyses were carried out in 21 patients with soft tissue sarcoma, all surgically treated in the same center, without chemotherapy or radiotherapy. In univariate analyses, DNA ploidy was found to be a significant factor for overall survival (OAS) and metastasis-free survival MFS. Mean OAS for aneuploid tumors and diploid tumors were 35 and 65 months (p=0.034), and mean MFS 23 and 61 months, respectively (p=0.005) .Discussion.There is a relation between histological grade and ploidy in soft tissue sarcomas. It appears that low-grade tumors are generally diploid, whereas high-grade tumors tend to be aneuploid. In a subgroup of patients treated only with surgery, DNA ploidy was found to be an important factor for predicting OAS and MFS.  相似文献   

2.
AIM: To evaluate the efficiency of our follow-up regime for patients with sarcoma of the extremities. METHODS: We have reviewed our follow-up policy in 480 patients with non-metastatic bone or soft tissue sarcomas and with more than 5 years of follow-up. RESULTS: We detected 24% of the soft tissue sarcoma recurrences and 38% of the bone tumour recurrences. We detected 67% of the pulmonary metastases before they became symptomatic. Only 11% of those patients who developed metastases were cured, as were only 26% of those who developed local recurrence. CONCLUSIONS: This study shows that our surveillance programme detects most metastases, but only about one third of the local recurrences.  相似文献   

3.
Sixty-five patients with soft tissue sarcomas and 40 patients with skeletal sarcomas were treated pre-operatively with infra-arterial Doxorubicin and radiation therapy (3500 rad in 10 fractions). The tumors were widely excised and in the case of skeletal sarcomas, the bone was replaced with a cadaver allograft or metal endoprosthesis in an attempt to achieve limb salvage. Chemotherapy consisting of Adriamycin and Methotrexate was administered post-operatively. These patients have been followed from 6 to 70 months with a median follow-up for soft tissue and skeletal tumors of 24 and 30 months respectively. In the soft tissue sarcoma group there have been 2 local recurrences; actuarial overall and disease-free survival at 3 years was 64 and 61%, respectively. In the skeletal sarcoma group there have been only 2 recurrences and the actuarial survival and disease-free survival of 75 and 55% at 2 years, respectively. In the soft tissue sarcoma group there have been 33 thigh lesions, 13 of which have required periosteal stripping. Five of the patients with thigh lesions have developed femoral fractures. All of these patients had post-operative chemotherapy and four of the five patients had periosteal stripping. In the skeletal sarcoma group, eleven of the twenty-one cadaver allografts have either fractured or become unstable requiring replacement with metallic endoprostheses that have been used exclusively for the past two years. Ninty-seven of the total group of 105 patients achieved limb salvage to the last follow-up or time of death with an acceptable complication rate. Longer follow-up is mandatory to determine the late effects of this combined modality regimen.  相似文献   

4.
目的探索侵犯关节周围骨组织的肢体软组织肉瘤的外科手术方法,并分析术后并发症、肢体功能和患者的生存状况。方法回顾性分析2004年5月至2011年10月期间,我中心所收治的30例局部侵犯关节周围骨组织的软组织肉瘤患者的临床资料。其中男14例,女16例,平均年龄51岁(17~75岁)。恶性纤维组织细胞瘤12例,脂肪肉瘤8例,原始神经外胚层肿瘤(PNET)4例,滑膜肉瘤、腺泡状软组织肉瘤和恶性神经鞘瘤2例。其中10例发病位于股骨近端,9例股骨远端,8例肱骨近端,2例胫骨近端,1例累及整个股骨。所有患者均采取了肿瘤连同受累骨组织一起广泛切除、以肿瘤型假体重建骨缺损的手术方式。术后定期复查患肢功能、x线片、肺CT等,并且密切随访,内容包括术后并发症的发生情况、肢体功能恢复情况及肿瘤学与生存状况等。结果平均随访25个月(3—84个月),1例暂时性腓总神经麻痹;3例术后伤口愈合不良行清创手术,其中1例因深部感染不愈而截肢;2例假体断裂行翻修手术;4例肿瘤复发,局部复发率13.3%,其中1例接受截肢手术。总的肺转移为15例,且11例死亡患者中均为肺转移,其中3例合并骨转移。至最后一次随访时14例无瘤生存,5例带瘤生存,平均MSTS评分股骨近端90%,股骨远端82%,胫骨近端73%,肱骨近端71%,全股骨为60%。2年和5年生存率分别为61.6%和30.8%。结论将受累骨组织和肿瘤一起广泛切除可获得无瘤边界,降低复发率,骨缺损采用肿瘤型假体重建可恢复良好的肢体功能。巨大肢体软组织肉瘤侵犯骨组织可能是患者预后不良的因素。  相似文献   

5.
BACKGROUND: The current study was performed to evaluate the toxicity profile of therapeutic doses of ifosfamide (IFX) given concurrently with full-dose external beam radiotherapy (EBRT) in patients with soft tissue and bone sarcomas. METHODS: The medical records of 43 consecutive patients with soft tissue or bone sarcomas who were treated with concurrent IFX and EBRT were reviewed. RESULTS: The median patient age was 20 years. Histologies were rhabdomyosarcoma (n = 16 patients), Ewing sarcoma (n = 10 patients), malignant fibrous histiocytoma (n = 9 patients), and other soft tissue sarcomas (n = 8 patients). Thirty-one patients (72%) had localized disease, and 12 patients (28%) had synchronous local and distant disease. Treatment consisted of EBRT (median dose, 50.4 gray [Gy]) with concomitant IFX (median dose per cycle, 10.2 g/m(2)). All patients with Ewing sarcoma or rhabdomyosarcoma received additional concurrent chemotherapy. Twenty-six patients (60%) received two or more cycles of IFX, and 17 patients (40%) were treated with one cycle of IFX and EBRT. The incidences of World Health Organization Grade 3 and Grade 4 toxicities were 29% (21 of 73 cycles) and 22% (16 of 73 cycles), respectively. Grade 4 systemic toxicities included leukopenia (n = 14 patients), neurotoxicity (suicidal ideation; n = 1 patient), and diarrhea (n = 1 patient). Confluent moist desquamation (Grade 3) occurred in nine patients in the treatment field; no patient experienced Grade 4 local toxicity. Among 14 patients who were treated preoperatively, 2 patients (14%) had a pathologic complete response, and 6 patients (43%) had a pathologic near-complete response (> or = 90% necrosis). CONCLUSIONS: Local and systemic toxicities after the administration of therapeutic doses of IFX with concomitant EBRT appear comparable to those observed with either treatment alone. These results support the design of prospective studies evaluating concurrent ifosfamide and radiation therapy for patients with sarcomas.  相似文献   

6.
BACKGROUND: The aim of this present report was to analyze the patients referred to us with the presumptive diagnosis of soft tissue sarcoma (STS). METHODS: We reviewed all patients referred to us with suspected soft tissue sarcoma (STS) of the extremities or trunk over a 12-year period. RESULTS: We treated 597 patients with soft tissue tumors. Open biopsy revealed soft tissue sarcoma in 318 cases, benign mesenchymal tumor in 124 cases and isolated metastases (ISTM) from carcinomas in 98 patients; other pathologies were found in 57 patients. The primary carcinomas were lung cancer in 26 patients, breast cancer in 19 patients, renal carcinoma in 16 patients, carcinoma of the esophagus in 12 patients, colonic carcinoma in 5 patients, thyroid gland cancer in 6 patients, and in 14 patients carcinoma of unknown primary was diagnosed. CONCLUSIONS: In our collective with soft tissue tumor, 50% of the patients had the diagnosis of soft tissue sarcoma, 20% presented with a metastasis of carcinoma and 20% had a benign tumor. Referring to our results, in patients with the presumptive diagnosis of soft tissue sarcomas, soft tissue metastasis of a primary carcinoma was unexpectedly common, indicating that greater consideration should be given to this differential diagnosis.  相似文献   

7.
Sarcomas of soft tissue and bone   总被引:5,自引:0,他引:5  
R Mazanet  K H Antman 《Cancer》1991,68(3):463-473
Sarcomas arise primarily from mesenchymal structures at any site in the body, even within visceral stroma and neurovascular bundles. Sarcomas have been associated with prior radiation therapy, toxic exposures, and genetic conditions and soft tissue sarcomas have been distinguished from bone sarcomas. For localized soft tissue sarcoma, tumor grade is the most important prognostic variable. Low-grade tumors are generally cured by wide surgical excision, but there is a significant rate of both local recurrence and development of distant metastasis in high-grade lesions. The treatment of soft tissue sarcoma histologic subtypes is generally similar grade-for-grade, with the exception of rhabomyosarcoma, Kaposi's sarcoma, and mesothelioma. Tumor location strongly influences resectability. Radiation therapy has been used successfully in conjunction with conservative surgery to improve local control rates for soft tissue sarcomas, particularly in extremity lesions. Currently, adjuvant chemotherapy remains unproven for most adult soft tissue sarcomas, but is established in the treatment of rhabdomyosarcomas, osteosarcomas, and Ewing's sarcomas.  相似文献   

8.
At the University of California Lawrence Berkeley Laboratory and the Northern California Oncology Group, a preliminary study of heavy charged particle radiotherapy in soft tissue and bone sarcoma has been carried out. Fifty-two patients with bone or soft tissue tumors were treated wholly or in part with heavy charged particles from 1978 to 1985. Eleven patients, considered inevaluable for purposes of this analysis, received less than 50 Gray-equivalents (GyE) because of the following: progressive disease (three patients); palliative treatment due to recurrent disease after previous radiation therapy (three patients); or since they were part of preliminary studies of relative biological effectiveness (RBE) (five patients). Forty-one patients received from 50 to 78.5 GyE, with a mean of 65 GyE. They had an average of 23 months follow-up, ranging from 4 to 78 months. In patients with paraspinal chondrosarcoma 9 of 11 had local control, with a mean follow-up time of 32 months. In the remaining patients with other histologies, 19 of 30 were controlled within the irradiated area, with a mean follow-up time of 20 months. Serious complications were encountered in the CNS (four patients), in the bowel (one patient), and in bone (one patient). Heavy charged particle radiotherapy appears to be of value in treating bone or soft tissue sarcoma; further trials are planned.  相似文献   

9.
From September 1984 to July 1987, 33 children received intra-operative radiotherapy as part of a multidisciplinary tumor treatment. Their age ranged from 2 to 17 years. Tumors types: Ewing's sarcoma (n=11), osteosarcoma (n=8), soft tissue sarcomas (n=5), Wilms' tumor (n=3), neuroblastoma (n=3), malignant pheochromocytoma (n=l), Hodgkin's disease (n=1), and optic nerve glioma (n=1). In 25 patients the disease was localized while 8 had distant metastases. Intraoperative radiotherapy was used in 26 previously untreated patients as part of a radical treatment program and in 7 cases as an effort to rescue local failures (5 in previously irradiated areas). The intraoperative radiation field included the surgically exposed tumor or tumor bed, and the single doses ranged from 10 to 20 Gy, with 6-20 MeV electrons. Patients with osteosarcoma and recurrent tumor in a previously irradiated area did not receive postoperative external beam radiotherapy. With a median follow-up time of 10 months (1 to 31+months) 24 out of 33 patients are alive without local recurrence and 9 have died from tumor (5 with local disease progression). Intraoperative radiotherapy seems to be a feasible treatment which might promote local control in pediatric tumors.  相似文献   

10.
PURPOSE OF REVIEW: This review focuses on the surgical management of soft tissue sarcomas of the hands and the feet. With recent advances in limb salvage surgery and radiotherapy delivery, local control of soft tissue sarcoma in the extremity has become optimized, and the associated functional results of this treatment have taken on extreme importance. Techniques to limit the amount of normal tissue resected and to reconstruct the resulting defects are critical to the final functional result. RECENT FINDINGS: Several features of soft tissue sarcoma unique to the hand and foot have been reported. Certain histologic subtypes of soft tissue sarcoma have been noted to arise preferentially in the hand and the foot, such as epithelioid sarcoma, clear cell sarcoma, and synovial sarcoma. Patients with hand and foot sarcomas have been described as having improved overall survival, but this is likely a result of the smaller size of tumors arising in these locations. Reconstruction of bone defects using various techniques, vascular reconstruction, tendon transfers, and soft tissue reconstruction using regional flaps in the hand and free flaps in the foot have resulted in good functional outcomes. Amputation and early prosthetic fitting still have a role in management of some soft tissue sarcomas, most frequently in the foot. SUMMARY: Limb salvage remains the standard of care for extremity soft tissue sarcomas. Given the fact that patients have good oncologic and functional outcomes with limb salvage in tumors in the hand and foot, surgical oncologists should have this goal for each patient.  相似文献   

11.
One hundred and seventy-one consecutive patients with soft tissue sarcomas were treated in the period 1977-1986. Of 144 patients with extremity sarcomas, only eight (6%) were managed with amputation. The overall estimated 5-year survival rate is 64%, and that for patients with extremity tumors is 71%. The 5-year local recurrence rate in extremity sarcomas was 6% for patients with minimum surgical margins 2 cm or greater and no further local therapy, and 6% for those with narrower surgical margins and adjuvant postoperative radiation; 80 patients (56%) were in the former group and 64 (44%) in the latter. With a selective combination of modalities, limb salvage can now be practiced in 94% of the patients with acceptable local control and survival rates.  相似文献   

12.
This is a retrospective study of 57 patients with extremity soft tissue sarcomas treated at Gujarat Cancer Research Institute, Gujarat, India from January 2005 to December 2007. It aims to review the feasibility of limb sparing surgery for extremity soft tissue sarcoma in Indian population. Twenty-eight percent (28%) of tumors were located in the upper limbs and 72% in the lower extremities. The goal of treatment was limb salvage if feasible. The treatment consisted of amputation in 17 (30%) and local wide excision i.e. limb salvage surgery (LSS) in 40 (70%) patients. The most common histology was pleomorphic sarcoma (21%). Sixty-four percent (64%) of the patients were treated with surgery alone, 32% with surgery plus radiation and 4% with surgery plus systemic chemotherapy. Inadequate margins were present in 12% of the patients. All the patients were followed up for at least 2 years. Recurrence in the form of local and distant occurred in 39% of the patients. Local recurrence occurred in 18% of the patients who underwent LSS and in 35% of those who underwent amputation. Pulmonary metastasis was detected in 16% of patients. The 2 year mortality was 10.5% with the most common etiology being lung metastasis and the most common histology being leiomyosarcoma. We concluded that limb salvage surgery for soft tissue sarcoma is feasible in the Indian population (70%) although the rate is lower than that of world literature at 90%. Keywords: soft tissue sarcoma, wide excision, amputation, limb salvage surgery, Indian population.  相似文献   

13.
BACKGROUND: The anatomic location of an extremity soft tissue sarcoma may influence the patient's presentation, management, and local and systemic recurrence rates. The objective of this study was to compare the presentation, management, and outcome of patients with soft tissue sarcomas of the upper extremity and the lower extremity. METHODS: Prospectively collected data from patients who underwent limb-sparing surgery for extremity soft tissue sarcoma between January, 1986 and April, 1997 were analyzed. Local recurrence free rates and metastasis free rates were calculated using the method of Kaplan and Meier. Univariate and multivariate analyses of potential predictive factors were evaluated with the log-rank test and the Cox proportional hazards model. RESULTS: Of 480 eligible patients, 48 patients (10.0%) had a local recurrence, and 131 patients (27.3%) developed distant metastasis. The median follow-up of survivors was 4.8 years (range, 0.1-12.9 years). Patients with upper extremity tumors had smaller lesions (6.0 cm vs. 9.3 cm; P < 0.001), more often underwent unplanned excision before referral (89 patients [64.0%] vs. 160 patients [46.9%]; P < 0.001), and less often received radiotherapy (98 patients [70.5%] vs. 289 patients [84.8%]; P < 0.001). The 5-year local recurrence free rate was 82% for patients with sarcomas of the upper extremity and 93% for patients with sarcomas of the lower extremity (P = 0.002). The 5-year metastasis free rate was 82% for patients with sarcomas of the upper extremity and 69% for patients with sarcomas of the lower extremity (P = 0.013). CONCLUSIONS: Local recurrence was more frequent in patients who had sarcomas of the upper extremity compared with patients who had sarcomas of the lower extremity. Factors that contributed to this difference included histologic type, the use of radiotherapy, and local anatomy. Metastasis was more frequent among patients with sarcomas of the lower extremity, because those tumors tended to be large and deeper compared with upper extremity tumors.  相似文献   

14.
Background and objectivesEwing sarcoma is the second most common bone sarcoma of childhood. Ewing sarcomas of the scapula are rare, with little known about their characteristics and outcomes. In this study, we describe the demographic characteristics, tumor characteristics, and oncologic outcomes of patients with Ewing sarcoma of the scapula.MethodsThis is a retrospective case series of thirty-four patients treated at three urban hospitals between 1993 and 2014 for Ewing sarcomas affecting the scapula. Their demographic data, tumor characteristics, and oncologic outcomes are reported and contrasted with data on Ewing sarcoma described in the literature.ResultsPatients in our case series were 59% male. The average age at diagnosis was 16 years. 44% of patients had metastatic disease at presentation. 26% of patients had a tumor size >8 cm in largest dimension at diagnosis. 9 patients in our series had the t (11; 22) translocation present. Patients had a survival rate of 68% at five years. No patients had local recurrence of disease. Compared with findings reported in the literature concerning Ewing sarcoma affecting other locations, patients with Ewing sarcoma of the scapula were slightly older at time of diagnosis, had a lower percentage of tumors with size > 8 cm in largest dimension at presentation, and more commonly had metastatic disease at presentation. Patients in our cohort had a 5-year survival rate of 68%, which is higher than the rate of approximately 55% as reported in the general literature.ConclusionsIn this study, we describe a retrospective case series of thirty-four patients with Ewing sarcomas of the scapula. This is the largest case series to date of Ewing sarcoma affecting this location to our knowledge. These results will contribute to the understanding of the clinical profile and oncologic behavior of Ewing sarcomas affecting the scapula.  相似文献   

15.
We report on the effects of intraarterial cisplatin and caffeine with/without doxorubicin on high-grade spindle cell sarcomas of bone and soft tissue based on the fact that caffeine enhances cytocidal effects of DNA-damaging agents. Intraarterial cisplatin and caffeine with/without doxorubicin was preoperatively given three times to ail patients and two courses of high-dose methotrexate with the citrovorum factor and vincristine were administered to the patients with skeletal spindle cell sarcoma. Tumor response was assessed radiographically and histologically. Seventeen (90%) of 19 patients with bone sarcoma and 7 (70%) of 10 patients with soft-tissue sarcoma showed good response. All patients with osteosarcoma demonstrating good radiological response underwent marginal excision without subsequent local tumor recurrence. Histologically, there were no viable cells in resected specimen of 14 patients with bone sarcoma. Other 8 cases with soft-tissue sarcoma treated by unplanned surgery were included to assess side effects. Twenty-five out of 37 patients are still free of disease. There was local tumor recurrence in 2 patients who did not respond to the chemotherapy. Toxic effects noted in the clinical study included moderate myelosuppression, nausea and vomiting, renal insufficiency and cutaneous injury. No toxic effects were directly attributable to 1.2-1.5 g/m(2) caffeine. The intraarterial infusion of cisplatin and caffeine combined with/without doxorubicin was tolerable in all patients and led to good response in high-grade spindle cell sarcomas. In patients with good response, limb-salvage surgery can be conducted safely without local relapse and good limb function is preserved by chemotherapy and marginal excision.  相似文献   

16.
T J Kinsella  E Glatstein 《Cancer》1987,59(5):908-915
Traditionally, adult bone and soft tissue sarcomas have been considered to be "radioresistant." Because of this philosophy, patients who present with locally advanced, unresectable sarcomas often are treated in a palliative fashion, usually with low-dose radiotherapy. Over the last 6 years, 29 patients with unresectable primary or metastatic sarcomas were treated using a combination of intravenous chemical radiosensitizers and high-dose irradiation. Twenty-two of 29 patients achieved clinical local control, with six patients having a complete clinical response. The time to tumor response is often several months or longer, which is in contrast to other tumor histologies (carcinomas, lymphomas), where tumor response usually occurs over several weeks. Several large tumors have shown only a minimal tumor response, yet were found to be sterilized in posttreatment biopsy or autopsy examination. Of 15 patients with primary sarcomas without metastases, 11 patients (73%) remain free of local tumor progression from 12 to 83 months. Adult high-grade sarcomas can be controlled with high-dose radiotherapy and intravenous radiosensitizers, although the precise role of these agents is unclear.  相似文献   

17.
A primary tumor arising in the hand or foot represents an uncommon presentation for patients with Ewing's sarcoma (ES) or soft tissue sarcoma (STS). While there exists considerable literature on the treatment of extremity sarcomas, very little deals specifically with lesions of the hand or foot. It remains controversial whether these lesions can be successfully treated with combined modality therapy which preserves the extremity and maintains function. From 1972 to 1979, 10 patients with sarcomas arising in the hand or foot were treated with combined modality therapy at the National Cancer Institute. Seven patients with ES of bone received local irradiation to 5000 rad and combination chemotherapy following an incisional biopsy. Three patients with STS received a gross tumor excision and local irradiation to 6000 rad. One STS patient also received combination chemotherapy. Local control was achieved in nine patients (90%) with a follow-up of 30-119 months (median 56 months). These patients have complete or almost complete function of the treated extremity. Nine patients are alive with five patients remaining disease-free following the initial combined modality treatment. Two patients with Ewing's sarcoma relapsed (1 patient with both local and distant failure) at 26 and 58 months and were again rendered disease-free with surgery, total body irradiation and further chemotherapy. One patient relapsed for a second time, being disease-free from the first relapse for 30 months. We conclude that for selected patients with sarcomas arising in the hand or foot, combined modality therapy which leaves the extremity intact results in excellent local tumor control and preserves function. Careful treatment planning is an essential aspect of successful radiation therapy of a hand or foot primary. Our treatment recommendations are outlined. This approach is a viable alternative to amputation in these patients.  相似文献   

18.
The results of surgical management of 63 cases of pulmonary metastases from bone and soft tissue sarcomas, admitted at the Istituto Nazionale Tumori of Milan, between 1970 and 1987, are reviewed in this paper. To estimate the relative impact of metastasectomy on the overall performance of treatment, survival curves were calculated from the time of first thoracotomy, as well as from the initial treatment of primary sarcoma. In the present series, total actuarial survival at 10 years was 37% for osteosarcoma, 27% for soft tissue sarcomas, and 24% for the other bone sarcomas, with a median survival of 48, 56, and 36 months, respectively. Five-year survival from the first pulmonary resection was influenced by the number of metastases and the length of the first disease-free interval only in osteosarcoma, while in soft tissue sarcomas a major untoward factor was represented by local recurrence at the site of the primary tumor. These data support the concept of pulmonary metastasectomy as effective salvage therapy for radically treated sarcomas; this management can rescue a significant proportion of all relapsed patients.  相似文献   

19.
To determine if amputation increases survival when compared to limb salvage surgery in patients with a soft tissue sarcoma (STS) of the extremity when there is often a misconception among physicians and patients that ablative surgery eliminates local recurrence and increases overall survival. This retrospective cohort study assessed 278 patients with STS and compared 18 patients who had undergone amputations for soft tissue sarcomas of the extremities to a comparative cohort of 260 patients who underwent limb salvage surgery during the same time period. Our limb salvage surgery (LSS) rate was 94% overall for soft tissue sarcomas with a median follow-up of 3.1 years. Patients undergoing amputations either had tumors that involved a critical neurovascular bundle (in particular nerve rather than vessel resection was more responsible for a decision toward ablation), or underlying bone or had neoplasms whose large size would require such an enormous resection that a functional limb would not remain. In comparing prognostic effects, mainly death due to sarcoma, distant metastasis and local recurrence, it was found that there was no statistically significant difference between patients undergoing amputation to those undergoing limb salvage surgery (p > 0.05). While amputations do not increase overall survival in soft tissue sarcomas of the extremity as compared to LSS, they are still a valuable option in a surgeon's arsenal. In particular, amputations can provide improved local control and symptomatic treatment in patients who might not be candidates for limb salvage surgery.  相似文献   

20.
Nonrhabdomyosarcoma soft tissue sarcomas form a group of rare tumors with a different biology and clinical behavior. The recently established European Pediatric Soft Tissue Sarcoma Study Group is organizing a new study devoted specifically to these tumors that were formerly treated according to the principles derived from experience with rhabdomyosarcoma, which is a clearly distinct entity. The new study includes two prospective trials, one for synovial sarcoma and the other for adult-type nonrhabdomyosarcoma soft tissue sarcomas. While surgery remains the mainstay of treatment, the role of adjuvant therapy is not yet clear and our understanding of the biology and natural history of nonrhabdomyosarcoma soft tissue sarcomas is still incomplete. This review presents the latest data on nonrhabdomyosarcoma soft tissue sarcoma treatment and outcome, and the rationale behind a risk-adapted treatment program that investigates the role of full-dose ifosfamide-doxorubicin chemotherapy in improving the response rate of patients with unresectable disease, the chances of avoiding adjuvant chemotherapy in low-risk synovial sarcomas, and the possible role of chemotherapy in high-risk adult-type soft tissue sarcomas.  相似文献   

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