间室切除术治疗四肢软组织肉瘤

 报告10例四肢软组织肉瘤行间室切除治疗的临床情况。仅2例为原发。平均随访19.6个月，复发2例（再发病例），转移1例。8例复发病例本次手术前平均复发时间2.2个月，本次术后平均复发时间为8.5个月，明显优于以前的治疗。认为，从控制肿瘤又保留肢体的观点出发，间室切除是一比较理想的术式.然而，其适应症比较窄，仅ⅡA和部分ⅡB者可选用。由于破坏性相当严重，常需同时使用功能重建和组织移植。本文还就与间室切除术有关的问题进行了讨论。     

影响软组织肉瘤预后的因素

 软组织肉瘤为较常见的恶性肿瘤，约占全部恶性肿瘤的0.8～1%。对于影响本病预后因素如肿瘤的大小、组织学类型、复发和转移等方面已得到公认。其他因素目前各家意见仍然不一，本文将以下几个因素作简要的综述。     

四肢软组织平滑肌肉瘤十例报告

四肢软组织平滑肌肉瘤临床少见。由于无特殊症状,临床常误诊为良性肿物而行单纯切除导致复发。我院自1987年11月～1990年11月共收治10例,现报道如下。     

体表复发性软组织肉瘤预后的有关因素及再治疗的探讨

目的：为了探讨影响体表复发性软组织肉瘤预后的的有关因素及再治疗问题。方法：收集我院１９７５－１９８５年间病理证实的复发性体表软组织肉瘤患者共１４１例，加予事理分析，其中包括１０个病理类型，治疗上采用单纯手术或手术加放疗和／或化疗为主。结果：３、５年生存率分别为５７．４５％和５１．７７％。结论：绝大多数复发性体表软组织肉瘤，综合治疗仍是最有效的措施。同时表明复发性肿瘤的预后还与病理类型、肿瘤大小、发     

14例软组织肉瘤综合治疗及预后因素分析

软组织肉瘤较少见,仅占全部恶性肿瘤1%左右,发病年龄32～54岁之间。四肢,躯干和腹膜后为常见部位。病变常呈隐匿性进展,可广泛侵犯局部组织,组织病理学和发病部位可以不同。现将本院1994年5月～2000年8月资料完整的14例软组织肉瘤治疗结果和预后因素做一回顾性报1.2临床分期根据1992年美国癌症联合学会(UICC)软组织肉瘤分期方法,Ⅰ期4例,Ⅱ期5例,Ⅲ期2例,Ⅳ期3例。1.3手术治疗14例中13例手术切除,经一次手术8例,经二次手术2例,三次手术3例,手术方式为肿瘤扩大切除术和肿瘤完整切除术。1例行切检术。1.4放射治疗14例均经过放射治疗,13例为…     

四肢和躯干软组织肉瘤辅助性近距离治疗进展

软组织肉瘤（soft tissue sarcoma,STS）是来源于问叶组织和周围神经组织的有明显异质性的恶性肿瘤,占成人全身恶性肿瘤的1％,50％～60％发生于肢体,但多数早期无明显症状而被忽视。诊断时肿瘤体积较大,并侵犯周围组织结构,治疗效果不理想,使人们认为软组织肉瘤是放射抗拒的。近年来,随着对软组织肉瘤生物学行为认识的不断提高,人们认识到其细胞对放射线的敏感性,放疗的应用逐渐推广,并占重要地位。     

腺泡状软组织肉瘤的临床和预后分析

目的探讨腺泡状软组织肉瘤(ASPS)的临床特点、治疗方法和预后情况。方法58例ASPS患者除6例就诊时即发现远处转移未行手术治疗外,其余52例均行手术治疗,其中19例行局部切除术,33例行扩大切除术。19例术后接受辅助性放疗或化疗。58例患者均获得随访,中位随访时间为52个月。结果50例肿瘤完整切除患者中,11例(22.0%)出现局部复发。全组58例患者中,31例(53.4%)发生肺转移。全组总的3、5和10年生存率分别为89.5%、74.1%和57.7%。中位生存时间为125个月。男性患者的3、5和10年生存率分别为79.6%、67.2%和49.7%,女性患者分别为100.0%、81.6%和65.3%(P=0.026)。结论ASPS恶性程度不高,肿瘤生长较为缓慢,局部复发率不高,但远处转移较为常见,肺是其最常见的转移器官。手术切除仍是目前治疗局限期ASPS的惟一有效手段。术后辅助性放化疗对控制局部复发和远处转移效果并不令人满意。女性患者的预后好于男性。     

软组织肉瘤局部复发的治疗及预后因素分析

采用单因素和多因素分析法 ,对 46例复发性软组织肉瘤的临床、治疗方法及其对预后的影响进行研究。本组低度恶性 (G1) 16例 ,中高度恶性 (G2 、G3 ) 3 0例。术式广泛切除 2 5例 ,局部扩大切除 8例 ,截肢 3例 ,术中放疗 16例 ,辅助化疗 2 3例。再次手术后 5年生存率为 5 8.2 % ,5年局部控制率为 69.2 %。强调对软组织肉瘤的首次合理治疗 ,广泛切除术的边缘应 >2cm ,局部复发肿瘤经积极综合治疗仍可获得较满意效果     

Genetic aberrations in soft tissue leiomyosarcoma

Leiomyosarcoma is a malignant mesenchymal tumor composed of cells showing smooth muscle differentiation. This tumor usually occurs in middle-aged or older adults, and forms a significant percentage of retroperitoneal, vascular, extremity, and uterine sarcomas. Leiomyosarcomas are most often associated with complex karyotypes with numerous chromosomal gains and losses. Some of these cytogenetic and molecular genetic aberrations correlate with histopathologic features and clinical outcomes. Identification of genetic alterations with specific identification of oncogenes and tumor suppressor genes may lead to additional insights into the tumorigenesis of leiomyosarcoma and the opportunity to confer the benefits of targeted therapy.     

Combined-modality treatment of localized soft tissue sarcomas of the extremities

Over the past 20 years, considerable progress has been made in the treatment of patients with extremity soft tissue sarcomas. There has been a migration away from amputation toward treatment by excision plus radiation for most patients with localized tumors. Decisions about the optimal use and sequencing of surgery and radiation remain complex. Whereas it is clear that local control is probably not impacted significantly by the treatment sequence, rates of wound complication, fibrosis, and edema are affected by the treatment sequence. In addition, recent single-institution reports indicate that some carefully selected patients can be treated by surgery alone. The recent data evaluating treatment by surgery alone and treatment sequencing variables do not lead to a situation in which clear, uniform recommendations for treatment can be made for many patients with extremity soft tissue sarcomas. Indeed, treatment planning for patients with extremity soft tissue sarcoma in the new millennium is infinitely more complex than it was in the era when amputation was the primary treatment for these patients. Considerable clinical experience and multidisciplinary input are required for optimal treatment planning for these patients. Future research should be directed at refining the indications for specific therapies, reducing the toxicities of local therapies, and developing more effective systemic therapies.     

Prognostic factors in soft tissue leiomyosarcoma of the extremities: a retrospective analysis of 42 cases.

AIMS: Soft tissue leiomyosarcomas (LMS) are rare tumours which are associated with a poor prognosis. The goals of the present study were to describe the clinico-pathological and prognostic features of 42 patients affected by soft tissue LMS of the extremities. METHODS: The following clinical and pathological parameters were analysed: age, sex, site, size, depth, previous surgical procedures, stage, histological type, nuclear atypia, grade, mitotic activity, necrosis, surgical margins, therapy. Disease-free survival rates were calculated. RESULTS: The overall 2-year and 5-year disease free survival rates were 42.3% and 32.6%, respectively. By univariate analysis, tumour size (< or =10 cm vs. >10 cm; p = 0.01), average mitotic rate (< or =19/10 HPF vs. >19/10 HPF; p = 0.05), type of excision (wide vs. marginal or intralesional; p = 0.001) and adjuvant radiotherapy (none vs. brachytherapy+external beam radiotherapy vs. external beam radiotherapy; p = 0.02) were significantly correlated with disease progression. By multivariate analysis, the only factor that was found to be an independent predictor of disease relapse was type of excision (p = 0.001). CONCLUSIONS: Large tumour size and high mitotic rate resulted adverse prognostic factors. Adjuvant radiation therapy, in combination with wide surgical excision, allowed the best chance of cure.     

Epigenetic signatures differentiate uterine and soft tissue leiomyosarcoma

Leiomyosarcomas (LMS) are diverse, rare, and aggressive mesenchymal soft tissue sarcomas. Epigenetic alterations influence multiple aspects of cancer, however epigenetic profiling of LMS has been limited. The goal of this study was to delineate the molecular landscape of LMS for subtype-specific differences (uterine LMS (ULMS) vs soft tissue LMS (STLMS)) based on integrated analysis of DNA methylation and gene expression to identify potential targets for therapeutic intervention and diagnosis. We identified differentially methylated and differentially expressed genes associated with ULMS and STLMS using DNA methylation and RNA-seq data from primary tumors. Two main clusters were identified through unsupervised hierarchical clustering: ULMS-enriched cluster and STLMS-enriched cluster. The integrated analysis demonstrated 34 genes associated with hypermethylation of the promoter CpG islands and downregulation of gene expression in ULMS or STLMS. In summary, these results indicate that differential DNA methylation and gene expression patterns are associated with ULMS and STLMS. Further studies are needed to delineate the contribution of epigenetic regulation to LMS subtype-specific gene expression and determine the roles of the differentially methylated and differentially expressed genes as potential therapeutic targets or biomarkers.     

High-grade soft tissue sarcomas of the extremities

From July 1975 to December 1982, 358 patients were referred to the Surgery Branch of the National Cancer Institute (NCI) with the diagnosis of soft tissue sarcoma of the extremities. Two hundred eleven of these patients presented with resectable, localized high-grade soft-tissue sarcomas and have been included in the present analysis of the management and outcome of patients with high-grade soft tissue sarcomas of the extremity treated at the NCI. One hundred forty-seven of these 211 patients have been included in randomized prospective trials. The remaining 64 patients in this analysis have been followed at the NCI, but were not included in randomized trials because of patient refusal or ineligibility. Tumor size was identified as a highly significant prognostic variable for disease-free and overall survival (P2 = 0.00001 and 0.0081, respectively). Tumor site, histologic type, and microscopic margins of resection were not significant prognostic variables. There was no difference between patients undergoing amputation compared to those undergoing limb-sparing procedures plus postoperative radiotherapy in disease-free or overall survival for all 211 patients in this study (P2 = 0.068 and 0.131, respectively). A significantly greater frequency of local failure among patients treated by local excision was noted compared to patients undergoing amputation (12/128 versus 0/83, P2 = 0.004), but this did not result in decreased overall survival in patients undergoing combined modality limb-sparing procedures. Adjuvant chemotherapy significantly prolonged disease-free survival (P2 = 0.005) for the 124/211 patients treated with adjuvant chemotherapy, although analysis of overall survival did not reveal a significant increase (P2 = 0.10). In a subset of 65 patients included in a prospective randomized trial evaluating the efficacy of adjuvant chemotherapy, a significant improvement in both disease-free (P2 = 0.033) and overall (P2 = 0.055) survival was seen in patients receiving chemotherapy. Sixty-five patients developed recurrent disease (65/211, 31%) and 42 of these patients were rendered disease-free surgically. Survival from the time of first recurrence was significantly prolonged among the 42 patients who were rendered disease-free (median survival, 31 months) compared to those who were not (median survival, 9 months, P2 less than 0.001).     

Management of soft tissue sarcomas of the extremities

Soft tissue sarcomas are a diverse and rare group of neoplasms often found in the extremities. Treatment is best carried out in regional cancer centers by a multidisciplinary group with a common interest in these disorders. Treatment is usually surgical, with radiation used in select cases to try to reduce the risk of local recurrence. The role of chemotherapy is controversial, but it may be useful in patients at highest risk of developing metastatic disease. The development of newer forms of targeted systemic therapy and techniques to reduce the morbidity from local treatment to the extremity are areas of intense research interest.     

Pathological evaluation of soft tissue sarcoma for diagnosis, prognosis and treatment

Soft tissue sarcomas (STS) are rare and heterogeneous group of tumors which differ widely in their clinicopathological features, and have a wide spectrum of clinical course, ranging from indolent tumors with a good prognosis to highly aggressive tumors with a poor prognosis. The diagnostic process of STS is complex and may necessitate an array of ancillary studies, including immunohistochemistry, electron microscopy and molecular genetic methods. Although an ongoing and improved histopathological definition of individual tumor types has been established, there still remains a subset of mesenchymal tumors which are currently not readily classifiable. This review summarizes our experience and that of others in the approach to the pathological evaluation of STS, and especially emphasizes the need to use emerging molecular techniques that can provide important clues for diagnosis, prognosis, and treatment of STS.