Role of chemotherapy in pediatric nonrhabdomyosarcoma soft-tissue sarcomas

The definition of nonrhabdomyosarcoma soft-tissue sarcomas includes a varied group of malignant soft part tumors that can occur in childhood, but the majority are entities typically observed in adult age. Similar to their adult counterparts, pediatric nonrhabdomyosarcoma soft-tissue sarcomas are usually considered scarcely sensitive to chemotherapy, but treatment strategies for these tumors have changed to some degree in recent years, and multiple-modality treatments that also include chemotherapy have increasingly been attempted. Subsets of patients with specific histological subtypes and prognostic variables have been thought likely to benefit from chemotherapy. The recent development of new molecular treatment approaches to specific tumor targets may enable the current limits of systemic therapies to be overcome in the near future, possibly identifying specific agents tailored to each histotype. While awaiting these developments, however, a better use of standard chemotherapy may prove important in improving the cure rate for these patients.     

Intraarterial adriamycin in the treatment of soft tissue sarcomas

Ten patients with extremity sarcomas adriamycin 60 mg/M² into the artery supp supplying the area of tumor. There were minimal local side effects consisting of occasional local erythema or slight transitory pain. Nine of these patients had subsequent surgery, and an average 32.86% histologic tumor necrosis was recorded in the peripherally viable areas of seven patients with residual tumor, compared to a 5.71 % necrosis recorded in the biopsy sections (P value < 0.01).     

New concepts for the treatment of pediatric nonrhabdomyosarcoma soft tissue sarcomas

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.     

Pediatric nonrhabdomyosarcoma soft tissue sarcomas

The nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) are a heterogeneous group of mesenchymal cell neoplasms that account for about 4% of childhood cancers. Because each histologic subtype of NRSTS is rare, they have been poorly studied and little is known about their biology, natural history, or optimal treatment. Data from adults with soft tissue sarcomas provide some helpful insight, but adult and childhood NRSTSs differ considerably in the distribution of their histologic subtypes, and certain entities are known to behave differently in young children. The greater risks posed to children by treatment, particularly by radiotherapy, also must be considered in treatment planning for children. This article summarizes what is known to date about childhood NRSTS, including the epidemiology, pathogenesis, and clinical approach to diagnosis and treatment of these tumors.     

Limb-sparing treatment for soft tissue sarcomas: Influence of prognostic factors

At present, limb-sparing surgery is the most appropriate and acceptable treatment available for sarcomas of the extremities, although the right balance between conservative therapy and maximum efficacy has yet to be found. A better knowledge of prognostic factors may help in planning the appropriate strategy for each case. Eighty patients underwent limb-sparing surgery for limb sarcomas (17 had surgery alone; 19 had neo-adjuvant hyperthermic antiblastic perfusion combined or not with postoperative radiotherapy, and 44 had adjuvant radiotherapy). Univariate and multivariate analyses were made to detect statistically significant differences between subgroups and identify the more significant subset of prognostic factors. Only microscopically positive surgical margins were related to a greater risk of local recurrence, whereas overall survival was compromised by high grade and large tumor size. © 1996 Wiley-Liss, Inc.     

Recent advances in systemic therapy of soft tissue sarcomas

Improvements in surgical techniques, for example reconstructive surgery; and radiation therapy techniques, such as intensity-modulated radiotherapy; have made some impact on the functional outcomes of sarcomas with a local biology. Effective local control can be achieved with effective function in the vast majority of patients. The problem of distant metastases, however, continues to plague a large group of patients with this disease. Recent advances in the systemic therapy of sarcomas is highlighted by the rapid development and approval of the molecularly targeted therapy imatinib (Gleevec^®) for advanced gastrointestinal stromal tumors. Several other agents have been, or are being studied with far less rewarding results. Among these, the more encouraging examples include the nucleoside analog gemcitabine (Gemzar^®) and gemticibine/docetaxel (Taxotere^®) in combination, which display some selective activity in sarcomas of gynecologic origin. The marine compound ecteinascidin (ET-743) has been studied in two different schedules (24 and 3 h infusions), demonstrating biological activity worthy of further investigation. Identification of new agents with activity in this diverse group of diseases is extremely important. Identification of specific targets responsible for tumorigenesis and effective inhibition of these targets holds the most promise for future improvement in cure rates. However, until such time, it is equally important to emphasize clinical research attempting to further optimize the use of the standard chemotherapeutic agents, with growth-factor support in appropriately selected patients.     

软组织肉瘤手术加术中放疗的临床研究

目的：探讨软组织肉瘤术中放疗的意义。方法：对39例软组织肉瘤患者行根治或姑息性手术，术中放疗在术中放疗手术室进行，术中根据肿瘤大小，选择不同术中放疗限光筒及6～12MeV电子线1次照射15～25Gy，姑息手术者剂量加大至36Gy。术后辅以外照射治疗，常规设野，5／周，2Gy／次，总量40～50Gy。初发病灶10例，术后复发29例。结果：39例患者随访12～64个月，3、5年局控率分别为71．8％和64．1％。3年生存率为82．0％。结论：术中放疗具有较高的局控率，比之其他治疗具有许多优点，将获得较高的生存率。     

软组织肉瘤手术加术中放疗的临床研究

目的:探讨软组织肉瘤术中放疗的意义。方法:对39例软组织肉瘤患者行根治或姑息性手术,术中放疗在术中放疗手术室进行,术中根据肿瘤大小,选择不同术中放疗限光筒及6～12MeV电子线1次照射15～25Gy,姑息手术者剂量加大至36Gy。术后辅以外照射治疗,常规设野,5/周,2Gy/次,总量40～50Gy。初发病灶10例,术后复发29例。结果:39例患者随访12～64个月,3、5年局控率分别为71.8%和64.1%。3年生存率为82.0%。结论:术中放疗具有较高的局控率,比之其他治疗具有许多优点,将获得较高的生存率。     

原发性256例软组织肉瘤手术治疗的临床分析

目的：探讨软组织肉瘤的诊断、手术治疗方法及其疗效。方法：对256例原发性软组织肉瘤患者的治疗情况进行回顾性分析。行局部广泛切除及根治切除术,对侵犯骨组织的软组织肉瘤则按微波原位灭活保肢手术处理。切除肿瘤后,行血管修复重建、带血管蒂游离皮瓣转移、局部皮瓣转移、肌腱移位、肌皮瓣移位。结果：发病年龄为20-70岁,占62.11%（159/256）,常见的病理类型为滑膜肉瘤、恶性纤维组织细胞瘤、脂肪肉瘤、横纹肌肉瘤、纤维肉瘤,占65.23%（167/256）。Kaplan-Meier法计算5年生存率为63.28%。结论：诊断采用CT、MRI为主的影像学,治疗以手术为主,辅助放疗和化疗。     

原发性256例软组织肉瘤手术治疗的临床分析

目的:探讨软组织肉瘤的诊断、手术治疗方法及其疗效。方法:对256例原发性软组织肉瘤患者的治疗情况进行回顾性分析。行局部广泛切除及根治切除术,对侵犯骨组织的软组织肉瘤则按微波原位灭活保肢手术处理。切除肿瘤后,行血管修复重建、带血管蒂游离皮瓣转移、局部皮瓣转移、肌腱移位、肌皮瓣移位。结果:发病年龄为20-70岁,占62.11%(159/256),常见的病理类型为滑膜肉瘤、恶性纤维组织细胞瘤、脂肪肉瘤、横纹肌肉瘤、纤维肉瘤,占65.23%(167/256)。Kaplan-Meier法计算5年生存率为63.28%。结论:诊断采用CT、MRI为主的影像学,治疗以手术为主,辅助放疗和化疗。     

野中野照射加大剂量顺铂水化防治软组织肉瘤术后复发的临床研究

目的评价野中野照射加大剂量顺铂（HD-PDD）水化技术在软组织肉瘤综合治疗中的地位及其疗效。方法127例软组织肉瘤术后患者随机分为研究组67例（野中野照射加HD-PDD水化）和对照组60例（常规照射、化疗）。研究组放射治疗：大野40～50 Gy,4～5周,小野20～30 Gy,3～4周,每天2野照射,间隔6 h;化疗：PDD 80～100 mg/m^2,每3周1次,共2～3次。对照组放射治疗：40～50 Gy,4～5周,后缩野加量20～30 Gy,2～3周。结果研究组和对照组1,3,5年生存率分别为95.5%和78.3%（P〈0.05）,82.1%和60.0%（P〈0.05）,70.2%和53.3%（P〉0.05）。研究组和对照组1,3,5年局部复发率分别为4.5%和10.0%（P〉0.05）,9.0%和31.7%（P〈0.05）,25.5%和41.7%（P〉0.05）。结论野中野照射加HD-PDD水化技术可在短时间内使肿瘤区接受较高剂量及获得放射治疗增敏,且明显提高了生存率,降低了局部复发率,作为术后综合治疗技术在软组织肉瘤治疗中具有重要价值。     

腺泡状软组织肉瘤的临床治疗分析

目的探讨腺泡状软组织肉瘤手术切除及辅助放、化疗的临床治疗效果。方法对1993～2006年间收治的腺泡状软组织肉瘤12例回顾性分析。男5例,女7例。年龄11～37岁。肿瘤直径平均5.5cm。结果12例手术后切除缘评价中,达到广泛切除缘者10例,边缘切除缘者2例,术后辅助放疗2例,全组均予辅助化疗。12例手术后复发3例,占25%。有7例发生转移,首诊时转移2例,其中肺转移瘤5例,脑转移瘤2例。转移率58.3%。转移瘤单纯化疗5例中PR3例,NC1例,PD1例,3例PR患者均采用MAI方案。随访时间10个月～10年6个月,12例中7例生存,5例死亡,经过Kaolan-Meire生存率计算,3年生存率81.2%,5年生存率52.8%。结论腺泡状软组织肉瘤外科治疗应以广泛切除术为基本原则,术后辅助放疗可以减少复发,辅助化疗是转移瘤的主要治疗方法。     

软组织肉瘤的放射治疗

软组织肉瘤(soft tissue sarcomas,STS) 是起源于结缔组织的软组织恶性肿瘤,具有多种不同类型。手术是 STS 主要治疗方法,放疗也是其重要的治疗方式并且是综合治疗早期选择之一。对 STS 进行放疗已经超过 50 年历史,术前和术后放疗对于局部控制都有疗效,只是不良反应不同。软组织肉瘤放疗技术包括远距离放疗(适形放疗、调强放疗、立体定向放疗等) 、近距离放疗(组织间插植放疗、腔内后装放疗、术中放疗等) 等。放疗技术的进步,提高了放疗的精准性和确定性,降低了对病灶周围正常组织的损伤。本文主要针对 STS 放疗技术以及适用原则进行综述。      

Pelvic soft tissue sarcomas

Pelvic soft tissue sarcomas (PSTS) are a rare, heterogeneous group of tumors. They have been usually analyzed with retroperitoneal sarcomas (RPS), but actually have key differences. Due to their unique anatomic location, symptomatic presentation of PSTS may be more common than RPS. Adequate imaging approach is paramount for guiding differential diagnosis, while preoperative biopsy is mandatory, especially when preoperative treatment may be considered as initial approach. The most frequent histologic subtype is leiomyosarcoma, which is different as expected in the retroperitoneum where liposarcoma is the commonest histology. Also solitary fibrous tumor is commonly diagnosed in the pelvis. Surgical approach for PSTS differs from that for RPS mainly due to anatomic relations. Similarly, in the lack of definite evidence from specific trials about neoadjuvant and adjuvant treatments, the anatomic constraints to obtain wide margins in the pelvis as well as the expected functional outcome in case of organ resections should be factored into decision for individualized treatment offer. Vascular and genitourinary involvement are frequent, as well as herniation through pelvic foramina. For these reasons a multidisciplinary surgical team should always be considered. Early referral of these patients to high-volume centers is critical and may impact on survival, given that optimal initial resection is a major predictor of curative treatment. International consensus on PSTS treatment is advocated, similarly to the recent efforts realized for RPS.     

Experience with 31 sentinel lymph node biopsies for sarcomas and carcinomas in pediatric patients

BACKGROUND: Few data exist regarding techniques, indications, and outcomes for sentinel lymph node biopsy in pediatric patients with sarcomas and carcinomas. METHODS: A retrospective 10-year review was conducted, with Institutional Review Board waiver, of the pathology, lymphoscintigraphy, and clinical records for all pediatric patients selected to undergo sentinel lymph node biopsy at a major cancer center. RESULTS: Thirty-one sentinel lymph node biopsies were performed in 30 pediatric patients (median age, 12 years; range, 2-21 years). With the administration of technetium 99m sulfur colloid, sentinel lymph nodes were identified preoperatively in 30 of 31 cases, and intraoperatively in the remaining case. Radiotracer alone was used in 13 of 31 cases but was supplemented with isosulfan blue dye in the remaining 18 cases. There were no complications. Positive sentinel lymph nodes occurred in 1 of 9 patients with rhabdomyosarcoma and in 2 of 5 patients with breast cancer, and in both of these diseases the sentinel lymph node results helped guide treatment decisions. No other patients had positive sentinel lymph nodes, and among those with nonrhabdomyosarcoma soft-tissue sarcomas there were no lymph node basin recurrences despite a lack of lymph node basin irradiation or formal lymph node dissection. The median follow-up was 48 months (range, 0-111 months). CONCLUSIONS: Sentinel lymph node biopsy for pediatric soft-tissue tumors can be performed safely, and the results can alter treatment decisions both for children with rhabdomyosarcoma and adolescents with breast cancer. In patients with nonrhabdomyosarcoma soft-tissue sarcoma, we observed no positive sentinel lymph nodes and no lymph node basin recurrences; these data should prompt the prospective study of sentinel lymph node biopsy as a modality that might help guide the administration or withholding of regional therapy among pediatric patients with nonrhabdomyosarcoma soft-tissue sarcoma.     

综合疗法防治软组织肉瘤术后复发的临床研究(野中野照射加大剂量顺铂水化技术的临床应用)

目的　评价野中野照射加大剂量顺铂(HDPDD)水化技术在软组织肉瘤综合治疗中的地位及其疗效。方法　63 例软组织肉瘤术后患者随机分为研究组32 例( 野中野照射加HDPDD水化) ,对照组31 例( 常规照射、化疗)。研究组放射治疗:大野40 ～50 Gy,4 ～5 周,小野20 ～30 Gy,3～4 周,每天2 野照射,间隔6 小时;化疗:PDD80～100 mg/m2 ,每3 周1 次,共2 ～3 次。对照组放射治疗:40～50 Gy,4～5 周,后缩野加量20 ～30 Gy,2 ～3 周。结果　1,2 ,3 年生存率分别为93.8 % 和77.4% ,84 .4% 和64 .5% ,81.3 % 和58.1 %( P<0 .05)。研究组局部复发率和远地转移率降低,但与对照组比较差异无显著意义( P> 0.05) 。结论　野中野照射加HDPDD水化技术可在短时间内使肿瘤区接受较高剂量及获得放射治疗增敏,且明显提高了生存率,降低了局部复发率和远地转移率,作为术后综合治疗技术在软组织肉瘤治疗中具有重要价值     

Emerging innovations and advancements in the treatment of extremity and truncal soft tissue sarcomas

In this special edition update on soft tissue sarcomas (STS), we cover classifications, emerging technologies, prognostic tools, radiation schemas, and treatment disparities in extremity and truncal STS. We discuss the importance of enhancing local control and reducing complications, including the role of innovative imaging, surgical guidance, and hypofractionated radiation. We review advancements in systemic and immunotherapeutic treatments and introduce disparities seen in this vulnerable population that must be considered to improve overall patient care.     

转移性软组织肉瘤化疗联合重组人血管内皮抑素治疗的有效性和安全性观察

目的 软组织肉瘤(soft tissue sarcomas,STS)是一种罕见的异质性恶性肿瘤,约80％患者术后两年内出现远处转移,且以多柔比星为基础的一线化疗,疗效依然不理想.本研究评价化疗联合重组人血管内皮抑素(恩度)治疗转移性软组织肉瘤的疗效及安全性.方法 回顾性分析天津医科大学肿瘤医院2007-01-01-2015-06-30收治的66例转移性STS病例,其中44例接受单纯化疗作为对照组,22例接受化疗联合恩度作为试验组.短期疗效评价包括客观反应率(objective response rate,ORR)、疾病控制率(diaease control,DCR)和安全性评价.随访观察无进展生存期(progression-free survival,PFS)和总生存期(overall survival,OS).结果 对照组和试验组ORR分别为13.6％和22.7％(x2=0.341,P=0.559),DCR分别为59.1％和77.3％(x2=2.135,P=0.144).对照组和试验组的中位PFS分别为6.9个月(95％CI为4.4～9.5)和12.5个月(95％CI为8.2～16.8),差异有统计学意义(x2=4.882,P=0.027),中位OS分别为15.4个月(95％CI为i0.2～20.7)和23.4个月(95％CI为16.4～30.3),差异有统计学意义,x2=4.506,P=0.034.对照组和试验组的1年生存率分别为58.1％和85.0％,2年生存率分别为24.6％和48.6％.大多数不良反应为轻到中度的呕吐,腹泻、骨髓抑制和心脏毒性,比较两组的不良反应差异无统计学意义,P＞0.05.结论 虽然化疗联合恩度没能控制转移性STS疾病进展,但是延长了患者的PFS及OS,且不良反应可以耐受.