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1.
AIM: Ten percent of soft tissue sarcomas (STS) arise in the retroperitoneal tissues. The prognosis for patients with retroperitoneal sarcoma is poor with a 5-year survival rate between 12% and 70%. Stage at presentation, high histological grade, unresectable primary tumour and incomplete resection are associated with a less favourable outcome. METHODS: Complete follow-up data were available on 22 patients who underwent surgery for retroperitoneal STS in our institution between 1990 and 2000. Patient, tumour and treatment variables were analysed including use of adjuvant therapy and survival status. RESULTS: Eighteen patients underwent surgery for primary disease, four patients were treated for recurrent disease or metastases. Ten patients presented with pain, seven with an abdominal mass, other presentation included weight loss and haematuria. Thirteen patients presented with tumours larger than 10 cm. The tumours were seven liposarcomas, six leiomyosarcomas, three malignant fibrous histiocytomas, two rhabdomyosarcomas, two malignant schwannomas and two undifferentiated sarcomas. Six primary tumours were completely excised, five patients received radiotherapy and five received chemotherapy. Local recurrence rate was 45% and recurrence-free interval for 10 patients with recurrence was 11 months. Five patients received radiotherapy and five received chemotherapy. The median survival for patients with primary tumours was 36 months, and 5-year survival was 44%. Adjuvant therapy was not associated with higher survival rates. CONCLUSION: This study re-emphasizes the poor outcome of patients with retroperitoneal STS. Adjuvant radiotherapy and chemotherapy do not appear to be any proven benefit and the single most important prognostic factor is aggressive successful en bloc resection of the primary tumour. Our resection rate and 5-year survival rates are comparable with previous reported UK series although lower than large reports from North American centres. This might partly be explained by difficulty in data collection in a retrospective analysis, but may reflect inadequate subspecialization in UK centres. Copyright Harcourt Publishers Limited.  相似文献   

2.
PURPOSE: To define the rate of local recurrence (LR) and identify prognostic factors for LR for patients with soft tissue sarcoma (STS) treated with function-sparing surgery (FSS) without radiotherapy (RT). PATIENTS AND METHODS: Between 1970 and 1994, 242 patients with STS of the trunk and extremity presented with primary localized disease, 74 of whom were treated with FSS without RT (31%). The median tumor size was 4 cm (range, 0.5 to 31 cm). There were 40 patients with grade 1 tumors and 34 with grade 2 and 3 tumors. Median follow-up was 126 months. RESULTS: The 10-year actuarial local control rate was 93% +/- 4%. Resection margin status was a significant predictor for LR. Patients with closest histologic resection margins of less than 1 cm had a 10-year local control rate of 87% +/- 6% compared with 100% for patients with closest histologic resection margins of >/= 1 cm (P =.04). There was no significant association between LR and tumor grade, size, site (truncal v extremity), or depth (superficial v deep). For all patients, the 10-year actuarial survival rate was 73% +/- 6%. CONCLUSION: The 7% LR rate after treatment of STS with FSS without RT reported herein is comparable to published rates following treatment where adjuvant RT is used. These results suggest there may be a select subset of patients with STS in whom carefully performed FSS may serve as definitive therapy and in whom adjuvant RT may not be necessary. However, further study is needed to carefully define this subset of patients and to identify the optimal surgical approach and technique for patients treated without RT.  相似文献   

3.
In more than one-third of patients with a histological high-grade malignant soft tissue sarcoma metastasis develops despite local control of the primary tumour. Hence, adjuvant chemotherapy is increasingly used for these relatively chemoresistant tumours which requires improved prognostication to exclude low-risk patients from overtreatment. We assessed the value of stepwise prognostication in a series of 434 histological high-grade STS of the extremity and trunk wall. Vascular invasion was used as the first discriminator whereafter the risk factors tumour necrosis, size (>8cm) and infiltrating growth pattern were used to discriminate high- and low-risk tumours. We identified a high-risk group with a cumulative incidence of metastasis >0.4 at 5 years, and a low-risk group, comprising half of the tumours, with a cumulative incidence of metastasis <0.15. The model was validated in an independent material of 175 patients. This model improved prognostication in STS and is of value for identifying patients who probably should not receive adjuvant chemotherapy.  相似文献   

4.
BackgroundAdult patients with advanced soft tissue sarcomas (STS) are generally treated similarly, regardless of great differences between STS subtypes, disease presentation and patients’ characteristics. As ifosfamide is frequently applied in first line systemic therapy, we aimed to establish prognostic and predictive factors for outcome to ifosfamide-based therapy.MethodsA retrospective, exploratory analysis was performed on data from 1337 advanced STS patients who received first-line ifosfamide-containing chemotherapy. For predictive factor analysis, 660 patients treated with doxorubicin monotherapy served as comparators.ResultsIndependent favourable prognostic factors for overall survival (OS) were good performance status, female gender, low histological grade, extremity primary tumour site and locally advanced disease; for progression-free survival (PFS), the combination of doxorubicin and ifosfamide, locally advanced disease, and tumour entity with a lower risk to progress for synovial sarcoma patients compared to leiomyosarcoma. For response, independent favourable prognostic factors were doxorubicin combined with ifosfamide, higher histological grade, and histology with synovial sarcoma patients having the highest chance to respond. Predictive factor analysis showed that compared to doxorubicin monotherapy, patients who benefited less from ifosfamide-based therapies were leiomyosarcoma patients in terms of OS, and patients with liposarcoma for response. No predictive factors were found for PFS.ConclusionIn this study, we established an independent set of prognostic and predictive factors for outcome to ifosfamide-based chemotherapy in advanced STS patients. This study provides important information for the interpretation and design of clinical trials for specific STS entities and may contribute to further treatment individualisation of advanced STS patients.  相似文献   

5.
肢体软组织肉瘤122例的临床分析   总被引:2,自引:2,他引:2  
目的 探讨肢体软组织肉瘤的临床治疗及预后因素。方法 对122例软组织肉瘤病例进行了回顾性分析,计算生存率并作单因素及多因素分析。结果 1、3、5及10年的总生存率分别为77.36%、66.90%、62.06%、54.22%。无瘤生存率1年为75.28%、3年为63.28%、5年为57.63%、10年为47.90%。Log-rank时序检验结果发现病理分级、病理类型、肿瘤大小、有无血行转移、侵犯血管与否、切缘、术前后辅助治疗等影响总生存率,而类型、分级、部位、大小、有无血管侵犯及血行转移、治疗方法及手术切缘影响无瘤生存率,差异有统计学意义。Cox回归分析结果显示病理分级、血管侵犯、有无转移是影响预后的主要因素。结论 肢体软组织肉瘤的治疗提倡广泛切除术,合理应用综合治疗及个体化治疗  相似文献   

6.
Purpose. Extraskeletal osteosarcoma represents an unusual soft-tissue sarcoma that historically is reported to carry an exceptionally poor prognosis.The objectives of this study were to use a prospectively gathered sarcoma database to test the prevailing clinical bias and more accurately describe the natural history, characterize the prognostic features, estimate survival and evaluate treatment strategies for this unusual sarcoma.Patients and methods. From a large database of nearly 4000 sarcomas at a single institution, 15 patients with pathologically confirmed extraskeletal osteosarcoma were analysed.Results and discussion. Extraskeletal osteosarcoma usually occurs as a large, deep, high-grade lesion in the lower extremity of older patients. Overall and disease-specific survival at 5 years was 50%, with a median follow-up of 35 months (range 3- 200 months). Use of adjuvant chemotherapy or radiation therapy did not appear to influence survival, but an effect may have been missed by the relatively low numbers in each group.When matched to a comparable group of patients with stage III extremity sarcomas, there was no significant difference in overall or disease-specific survival between groups. Treatment for extraskeletal osteosarcoma should follow established guidelines for treatment of soft-tissue sarcomas, with the decision regarding adjuvant therapy to be based on individual risk factors.  相似文献   

7.
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   

8.
Adjuvant therapy for sarcomas.   总被引:2,自引:0,他引:2  
Adjuvant therapy is currently established in the treatment of osteosarcoma, Ewing's sarcoma and rhabdomyosarcoma. Of the 12 reported randomized studies of adjuvant chemotherapy for soft tissue sarcoma, only 2 show a significant overall survival advantage for chemotherapy (the most important endpoint). In three randomized trials, the survival of the observation arm exceeds that of the chemotherapy arm. In two additional studies, subset analyses currently indicate a significant DFS advantage for adjuvant chemotherapy in extremity lesions, but no significant improvement in survival. Although initial NCI reports showed significantly prolonged survival for the subset of chemotherapy-treated extremity primaries, survival on longer follow-up is no longer significantly different. In the subset analysis of retroperitoneal sarcomas in the same NCI study, the survival of the control group is superior to the treatment group. Doxorubicin associated cardiotoxicity has occurred in about 10% of treated patients, occasionally contributing to treatment-related deaths. Based on these data, adjuvant chemotherapy should be considered investigational for adult soft-tissue sarcomas of any primary site. Future randomized trials should include patients at high risk for metastases (large, high-grade lesions) with a reasonable likelihood of local control by radical resection, or resection with uninvolved margins and subsequent radiotherapy. Low-grade sarcomas are currently cured by surgical resection in 80% of cases, and thus should not be included in adjuvant trials.  相似文献   

9.

Aims

Limb-sparing surgery combined with radiotherapy (RT) is the basis of extremity soft tissue sarcoma (STS) treatment. The aim of this study was to evaluate the results of microvascular reconstruction after extensive tumour resections in the upper extremity.

Methods

Twenty patients with STS of the upper extremity were treated with excision and microvascular reconstruction. RT was administered if surgical margins were less than 25 mm. Results were evaluated retrospectively.

Results

Twenty free flaps were performed and no flaps were lost. There was no operative mortality, and wound complication rate was 15%. Median follow-up length was 74 months. Five-year local recurrence-free survival was 57%, metastasis-free survival 67%, disease-free survival 45% and disease-specific overall survival 80%. Ten patients had no or only mild impairment of upper extremity function, seven had impaired function affecting daily life, and three patients underwent amputation. For patients treated with curative intent, limb salvage rate was 94%.

Conclusions

Free flaps are useful and reliable in the treatment of patients with STS of the upper extremity. Without microvascular reconstruction limb salvage would have been impossible in these patients. Oncological outcome is comparable to other extremity STS patients and upper extremity function is acceptable.  相似文献   

10.
Local management of adult soft tissue sarcomas   总被引:1,自引:0,他引:1  
Soft tissue sarcomas (STS) are rare tumors classified into multiple histological subtypes and categorized into four sites: extremity and trunk, head and neck, retroperitoneal, and visceral, the latter now predominantly consisting of gastrointestinal stromal tumors. Well-planned, complete surgical resection is the mainstay of curative therapy for tumors at each of these sites. The success of surgery alone in controlling disease varies with the site, histologic grade, depth, and size of the tumor. For high-risk tumors, adjuvant therapy should be considered. In high-risk extremity tumors, adjuvant radiation has been proven in randomized trials to improve local control. Limb-sparing surgery combined with adjuvant radiation achieves equivalent local control to amputation, with the same distant relapse-free survival. Due to anatomical constraints, tumors of the head and neck and retroperitoneum are typically excised with close margins, providing a rationale for adjuvant radiation; the available evidence suggests but does not prove a benefit. Large-scale trials of adjuvant imatinib for gastrointestinal stromal tumors are currently being conducted. For tumors of the extremity/trunk, head and neck, and retroperitoneum, biopsy prior to definitive resection is recommended to establish the diagnosis and permit intelligent treatment planning with appropriate choice and sequencing of adjuvant therapies. This planning is most expeditiously done through multidisciplinary consultation at an experienced sarcoma center.  相似文献   

11.
Seventy-eight patients with soft tissue sarcoma (STS) arising in the distal extremities--wrist, hand, finger, ankle, foot, and toe--who were treated with conservation surgery and radiation therapy were studied retrospectively with respect to survival, local recurrence, functional limb preservation, complications, and distant metastasis. After a median follow-up of 7.9 years, actuarial 5-year and 10-year survival rates were 80% and 69%, respectively, and disease-free rates were 61% and 51% at the same times. Actuarial local control rates were 80% and 74% at 5 and 10 years, respectively. Fifteen patients (19%) had local recurrence, but 12 of these were salvaged. Ultimately, 53 patients (68%) retained a normal or fairly normal extremity, six (8%) needed amputation for complications, and 13 (17%) needed amputation to control recurrent disease. The functional outcome was significantly better for patients with upper extremity lesions than for those with lower extremity tumors; even for the latter, this treatment strategy was preferable to amputation. The incidence of hematogenous metastases from distal extremity sarcomas depends on the size of the primary tumor. It was concluded that conservation surgery and radiation therapy (XRT) is an acceptable treatment strategy for STS arising in distal extremities; it yielded a high rate of disease control and functional limb preservation.  相似文献   

12.
From July 1975 to February 1985, 300 patients with extremity soft tissue sarcomas (STS) were entered into treatment protocols at the National Cancer Institute. Using the Cox proportional hazards model, the STS were analyzed for independent prognostic variables that had significant association with disease-free and overall survival. The effects of adjuvant treatment were also included in the analysis. Percent tumor necrosis was the basis for separating intermediate and high-grade STS (less than 15% necrosis = grade 2; greater than 15% necrosis = grade 3). The most important prognostic variable was histologic grade, which was highly associated with relative risk of metastasis and fatal outcome (P less than .0001). Histologic type of STS was not a significant prognostic variable in predicting metastases. Patients with tumors located on the proximal or middle part of the extremity had a fivefold increased risk of death (P = .02) compared with patients who had STS located more distally. Mitotic activity (P = .0005) and vascular invasion (P = .0034) were also associated with malignant behavior, but histologic grade was the most significant predictor of outcome. Histologic grade of malignancy was based upon a combination of tumor typing and visual estimation of extent of tumor necrosis; using these two features, one can reliably separate low-grade (i.e., grade 1) from high-grade STS and also stratify the more aggressive (i.e., grade 2 and 3) tumors. Over the 9.5-year period of study, the tumor-related mortality was as follows: grade 1 (1/38), 3%; grade 2 (19/142), 13%; and grade 3 (45/120), 38%.  相似文献   

13.
Randomized clinical trials in soft tissue sarcoma   总被引:3,自引:0,他引:3  
What has been learned thus far from the very best prospective randomized controlled clinical trials for soft tissue sarcoma that will guide physicians in the quest for evidence-based treatment decision making? Based on the evidence provided in the following articles, we have learned that limb-sparing surgery with adjuvant radiation is equivalent (and therefore preferable) to a radical amputation in controlling local disease. We also learned that adding radiation therapy (both external beam and brachytherapy) to a complete surgical resection of extremity soft tissue sarcoma significantly improves local control over surgery alone. This is true for high-grade lesions and may be true (external beam) for larger low-grade lesions also. Local control, however, does not govern overall survival as most patients who die from this disease do so from systemic (not local) manifestations. Finally, further studies are required to identify the role of adjuvant chemotherapy in the treatment of soft tissue sarcoma as no single study has demonstrated a consistent long-term survival benefit with the use of any chemotherapeutic regimen. Based on a recent large meta-analysis, however, if there is any benefit to adjuvant doxorubicin based chemotherapy, it must be a very small one.  相似文献   

14.
The management of clear cell sarcoma.   总被引:4,自引:0,他引:4  
Clear cell sarcoma is a rare soft tissue tumour, constituting approximately 1% of all soft tissue sarcomas. Prognosis is reported to be poor due to the great propensity to metastasise regionally and distantly. In this paper, we report the surgical experience of two university hospitals. Both disease-free and overall survival after resection of clear cell sarcoma in this limited study were excellent, which may be explained by relatively small tumour size in seven out of eight patients and adjuvant radiation treatment. The current treatment for clear cell sarcoma is wide local tumour excision, with adjuvant radiation therapy for resection margins of less than 1 mm.  相似文献   

15.
Data on 145 cases of soft-tissue tumor of the trunk were analysed. Tumor was located on the chest or back in 81 patients and deep in the anterior abdominal wall--in 64. When intramuscular sarcoma is removed, a 2-3-cm margin should be left around the palpable mass. Five-year survival in the entire group of patients with tumors of the chest and back was 59.4% +/- 2.4. Five out of ten cases with tumor of the abdominal wall survived over 5 years.  相似文献   

16.
In this phase II study, activity and safety of neoadjuvant regional hyperthermia (RHT) combined with chemotherapy was investigated in 59 patients with primary advanced or recurrent high-risk soft-tissue sarcoma (STS). Patients received four EIA cycles consisting of etoposide, ifosfamide and doxorubicin combined with RHT followed by surgical resection and adjuvant treatment. The overall objective response (OR) rate was 17%, with one complete (2%) and eight partial (15%) responses. In addition, 13 minor responses (25%) were seen. At time of surgery, complete necrosis (pCR) occurred in 6 patients and >75% necrosis (favourable histological response (FHR)) in 12 patients. At the completion of protocol treatment, 36 patients were rendered disease-free which was significantly associated with the initial radiographic and/or pathological tumour response (P=0.004). Treatment-related toxicity was acceptable overall. At a medium follow-up of 82 months, local treatment failure occurred in 33 patients, median overall survival (OS) was 52 months, and the 5-year survival rate was 49% (95% confidence interval (CI): 36-61%). OS which did not differ for extremity versus non-extremity STS (P=0.21) was better for patients responding to EIA combined with RHT (P<0.01).  相似文献   

17.
From 1975 to 1988, 144 patients naive of treatment, with non-metastatic soft tissue sarcoma were treated at Fondation Bergonié by surgery, followed by radiotherapy and without chemotherapy. An analysis of prognostic variables was done on this population to determine patients for whom an adjuvant chemotherapy would be relevant. Prognostic variables in overall survival (OS), metastasis-free survival (MFS), disease-free and local free recurrence survivals were analysed by univariate and multivariate analysis. In multivariate analysis using Cox's model, only tumour depth and tumour grade were significant with the MFS end point, while tumour depth, tumour grade and tumour site were significant when considering OS. A predictive stratification for patients is proposed: a favourable prognostic group with grade 1 tumour or superficial, grade 2 tumour (5-year OS: 97.8%; 5-year MFS: 100%); an intermediate prognostic group with deep, grade 2 tumour or superficial, grade 3 tumour (5-year OS: 58.8%; 5-year MFS: 48.1%); and finally a poor prognostic group with deep, grade 3 tumour (5-year OS: 31.7%; 5-year MFS: 34.1%). Patients in the intermediate and poor prognostic groups who present a high metastatic risk are to be considered for adjuvant chemotherapy trials.  相似文献   

18.
Adjuvant radiotherapy (RT) is routinely recommended for most soft-tissue sarcomas (STS) of the extremities. However, its impact on local control is not clearly established after wide complete excision. We performed a retrospective analysis of patients who underwent wide resection in our institution (first or second resection in cases of incomplete surgery) and either did or did not receive adjuvant RT. All histological specimens of patients operated upon between 1975 and 1996 were carefully analysed and only patients with free tumour margins (ftm) were retained for the analysis. The histopathological classification was as follows: minimal resection (mR) (ftm<10 mm) and optimal resection (oR) (ftm >/=10 mm). There were 133 patients with a median age of 44 years (range 16-88 years). The median tumour size was 6 cm (range 1-20 cm) with 28, 44 and 28% of stage I, II and III lesions, respectively. 93 patients (70%) were reoperated upon and residual tumour was found in 55% of the patients (51/93). 69 patients (17 oR and 52 mR) received adjuvant RT and 64 patients did not (54 oR and 10 mR). Other patient characteristics (age, tumour size, stage, deep-seated lesion, histoprognostic grade, adjuvant chemotherapy) were similar in both the RT and no-RT groups. Median follow-up was 10 years (3-25 years). The 5- and 10-year local relapse-free survival rates were 78 and 71%, respectively. 33 patients relapsed locally: 11 in the RT group and 22 patients in the control group (P=0.01). In the univariate analysis, adjuvant RT was correlated with relapse-free survival, while tumour grade and tumour margin status were correlated with overall survival. The multivariate analysis demonstrated a favourable impact of RT and negative influence of malignant fibrous histiocytoma (MFH) on local relapse-free survival; the tumour grade was correlated with overall survival. RT had a positive influence on local control exclusively in patients with mR resection (P=0.005) and in patients with residual tumour cells after re-excision (P=0.001). RT had no influence on 5- and 10-year overall survival. The 5- and 10-year overall survival for the entire population were 77 and 67%, respectively. Optimal resection seems to be the best predictive parameter for a favourable outcome in localised STS. Adjuvant RT is indicated after mR resection and for residual tumour after definitive surgery, but its role after oR resection (primary resection or no residual tumour after re-excision) should be evaluated in a prospective randomised trial.  相似文献   

19.
  目的  分析软组织肉瘤(soft tissue sarcoma,STS) 远隔软组织转移患者的临床特点、预后影响因素及不同治疗手段的疗效差异,为其临床诊疗提供参考。  方法  收集 2010 年 6 月至 2016 年 6 月北京大学肿瘤医院骨与软组织肿瘤科收治的发生远隔软组织转移的 STS 患者 25 例。统计分析其临床病理特点及预后情况。  结果  25 例远隔软组织转移患者中同时性转移 6 例(24%) ,异时性转移者 19 例(76%) ,原发灶手术后到出现转移病灶的平均时间为 45.3 个月。转移灶最常见的部位是躯干,共 18 例(72%) 患者,其次为头颈部 5 例(20%) 。本组患者 1 年总生存率 80%,3 年总生存率 33%,11 例(44%) 伴有肺转移患者 3 年总生存率较差,差异具有统计学意义。  结论  STS 远隔软组织转移患者中组织学分级 2 级、无肺转移者有更高的总生存率;新辅助化疗、手术为主的综合治疗是主要治疗方案,靶向治疗作为新的治疗手段呈现较好的疗效。    相似文献   

20.
AIMS: To identify independent adverse clinico-pathological factors for disease-free and overall survival in patients undergoing isolated limb perfusion (ILP) with cisplatin and doxorubicin for locally advanced soft tissue sarcoma (STS) of an extremity. METHODS: A retrospective analysis was carried out, using a univariate method and a multivariate analysis, to look at the patient, tumour and treatment associated with prognostic factors in 37 patients with locally advanced STSs of the limbs who underwent ILP with cisplatin and doxorubicin. Patient's age, gender, presenting status, tumour location, tumour grade, tumour stage according to TNM classification, tumour size and radiotherapy (RT) were analysed. Survival curves were calculated according to the Kaplan-Meier method. A Cox proportional hazard model was used to indicate which factors related to overall survival and the recurrence-free interval after ILP. RESULTS: No major systemic toxicity was seen. Regional toxicity was limited. Limb salvage was achieved in 94.6% of the patients. The estimated 5-year overall and disease-free survival rates were 62% and 54%, respectively. It was found that tumour stage, tumour grade, presenting status, RT and tumour size were associated with cumulative overall survival when the Kaplan-Meier method was applied (P<0.05). By Cox proportional hazards model, only tumour grade (P=0. 0254) was found to have significant influence on overall survival; however, tumour stage (P=0.0157) and RT (P=0.0014) were related to disease-free survival. CONCLUSIONS: ILP and delayed excision followed by RT achieves good limb salvage rates and may improve survival.  相似文献   

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