Ewing氏肉瘤综合治疗分析

Ewing氏肉瘤是一种少见的骨原发肿瘤 ,临床治疗以综合治疗为主 ,现回顾性分析从 1979年— 1995年本院收治的2 0例 Ewing 氏肉瘤 ,并结合文献对Ewing氏肉瘤的特点和现代综合治疗方案进行探讨。1　材料和方法1979年 6月— 1995年 6月本院共收治 Ewing氏肉瘤 2 0例 ,年龄分布 3岁～ 2 6岁 ,中位年龄 14岁 ,原发于股骨 4例 ,尺骨 2例 ,骨盆 2例 ,脊椎骨 4例 ,锁骨 2例 ,肋骨 6例。其中 4例诊断时出现肺转移 ,全部病例均经病理证实。 6例单纯手术切除 ,10例给予局部 (病灶去除术加放射治疗 ,而 4例给予局部放射治疗 ,共有 6例病例给予辅助化疗 …     

Ewing氏肉瘤的研究进展

以往Ewing氏肉瘤治疗的5年生存率为10％,且大多数病例死于转移性病变。本文旨在复习影响Ewing氏肉瘤的预后因素、生存时间以及采用现代联合治疗方法后的复发情况。此外,对放疗诱发肿瘤的问题也作简要讨论。影响Ewing氏肉瘤预后的因素Ewing氏肉瘤原发病灶的位置对预后影响极大。研究显示,发生在骨盆的Ewing氏肉瘤预后最差。Bacci等对144例骨盆Ewing氏肉瘤随访5年以上,其5年生存率为23％,而其它部位的Ewing氏肉瘤为46％。欧洲Ewig氏肉瘤合作研究中心(CESS)对93例局限性Ewing氏肉瘤施行化疗、放疗和手术治疗。随访结果显示,肿瘤容积是影响预     

18例骨外Ewing肉瘤的临床分析并文献复习

背景与目的:骨外Ewing肉瘤是指原发于骨组织外的尤文氏肉瘤,发病率低。本研究旨在通过对骨外Ewing肉瘤进行临床分析,探讨其临床特点、诊断和治疗方法。方法:回顾分析中山大学肿瘤防治中心1995年1月～2007年7月确诊并接受治疗的18例骨外Ewing肉瘤患者的临床资料,同时结合文献加以讨论。结果:18例患者中,男性13例,女性5例;年龄8个月～60岁,中位年龄17岁,其中12例患者(66.7%)年龄为5～25岁。肿瘤原发于下肢8例(44.4%),椎旁5例(27.8%),胸壁2例(11.1%)。临床症状表现为肿块16例(88.9%)。16例患者接受了综合治疗,2例接受了非综合治疗。1、3、5年生存率分别为82.4%、64.2%和32.1%。单因素分析显示有无远处转移及治疗模式与预后相关。结论:该肿瘤好发于青少年,主要表现为局部肿块,采取积极的综合治疗方案是主要治疗方法,预后与有无远处转移及治疗模式相关。     

氨磷汀联合化疗治疗Ewing肉瘤/PNET的临床观察

目的探讨氨磷汀联合化疗治疗Ewing肉瘤/PNET的疗效、不良反应和安全性。方法 32例Ewing肉瘤/PNET患者分为化疗加氨磷汀组(观察组)及单纯化疗组(对照组),观察组12例,对照组20例。行IFO-IFO-DDP-ADM化疗2周期后评价疗效、不良反应。结果观察组完全缓解(CR)及非常好的部分缓解率(VGPR)为91.6%,对照组为90.0%,两组比较差异无统计学意义(P>0.05)。比较两组各种Ⅰ度～Ⅳ度不良反应发生率,差异无统计学意义(P>0.05)。比较两组发生的Ⅳ度不良反应,观察组Ⅳ度白细胞减少为25.0%,对照组为70.0%;观察组Ⅳ度粒细胞减少25.0%,对照组为75.0%,差异均有统计学意义(P<0.05)。结论氨磷汀联合化疗治疗Ewing肉瘤/PNET不改变化疗疗效,不增加各种常见不良反应发生率;可以明显减少化疗后Ⅳ度白细胞及粒细胞减少的发生率,安全性好。     

Ewing肉瘤知识问答

Ewing肉瘤(尤文肉瘤或尤因肉瘤)是一种多发于儿童和青少年的恶性肿瘤.但由于较为罕见,许多人对它了解并不多.今天,咱们就一起通过问答方式来了解一下Ewing肉瘤的相关知识. 1.什么是Ewing肉瘤? Ewing肉瘤,以前被称为原始神经外胚层肿瘤(PNET),是一种来源于骨或者软组织的恶性肿瘤,整体人群发病率约百万分之一,好发于儿童和青少年,新生儿和婴儿病例偶有报道.     

骨外软组织Ewing肉瘤的临床病理学分析

背景与目的：骨外软组织Ewing肉瘤是临床上十分罕见的高度恶性肿瘤，报告2例病例并探讨其临床病理学特征。方法：运用光镜和电镜观察、组织化学及免疫组化染色方法，并复习以往相关文献。结果：骨外软组织Ewing肉瘤瘤细胞呈小圆形，异型性明显，较少或无菊形团（Homer-wright）结构。PAS特殊染色胞质中大量阳性细小颗粒沉积。免疫组化CD99（MIC2）和Vimentin标记呈强阳性表达。电镜观察可见瘤细胞质内有少量神经内分泌颗粒及丰富的糖原颗粒分布。结论：骨外软组织Ewing肉瘤是Ewing／pPNET家族肿瘤的一个亚型。骨外软组织Ewing肉瘤具有高度恶性、易复发和转移的临床特点，若出现神经分化的特点可能是具有更高侵袭性和不良预后的标志。     

儿童骨肿瘤的计算机断层摄影

作者用CT检查27例儿童骨肿瘤患者,报导了CT诊断的价值及其适应症。27例肿瘤中恶性22例(骨15例、软组织7例):Ewing氏肉瘤9例、骨生肉瘤3例、横纹肌肉瘤2例、纤维肉瘤1例、血管内皮肉瘤1例、神经母细胞瘤1例、转移瘤3例、滑膜肉瘤2例,另良性肿瘤5例。举出9个典型病例,如一例平片难于看到的骶骨Ewing氏肉瘤,CT表明有骶骨的破坏及骶前包块;一例股骨Ewing氏肉瘤从CT看出正常骨与肿瘤的分界,较平片与骨放射性同位素检查更清楚,经手术证     

髋臼周围肿瘤切除术后重建

目的探讨对髋臼周围肿瘤手术切除及重建骨盆稳定性的方法，最大限度保留肢体功能，减少复发。方法2002～2005年15例髋臼周围肿瘤患者，其中软骨肉瘤8例，恶性纤维组织细胞瘤3例，Ewing肉瘤2例，转移瘤2例；6例配合化疗，4例行瘤段灭活再植，1例行瘤段灭活再植＋人工全髋置换，10例行人工假体置换（包括全髋）。结果1例术后死亡，1例感染，清创后股骨头旷置；14例术后随访9～33个月，4例软骨肉瘤复发，其他病例均能保留患肢部分功能和骨盆稳定性。结论恶性纤维组织细胞瘤、Ewing肉瘤化疗有效者保肢率高，局部复发率低；软骨肉瘤复发率高；瘤段灭活再植因骨愈合时间长，影响功能；股骨头旷置肢体短缩，跛行明显；人工假体置换可最大限度地保留肢体功能；单纯转移瘤为提高生存质量，减轻痛苦，可考虑手术治疗，但年龄大，全身脏器应急能力差者，手术应慎重。     

罕见病-去分化软骨肉瘤的研究进展

去分化软骨肉瘤是一种罕见的高度恶性的原发性骨恶性肿瘤,除了含有高分化软骨肉瘤或交界软骨病损,还出现一种或多种高度恶性(Ⅲ-Ⅳ级)其它恶性肿瘤成分,如纤维肉瘤、恶性纤维组织细胞瘤、骨肉瘤、平滑肌肉瘤、未分化肉瘤及/或横纹肌肉瘤等,目前对该病的组织学来源仍然存在争议,主要临床表现为局部疼痛和软组织肿块,术前准确诊断是影响生存率的重要因素之一,术前诊断主要依赖影像学检查,其影像学表现为:骨皮质反应、骨皮质破坏、病理性骨折、骨内基质钙化、巨大软组织肿块及双相肿瘤等.最重要的影像学特征是双相肿瘤改变.确诊主要依靠术后组织学.治疗为以手术为主的综合治疗,至今没有任何统计学证据表明术前新辅助化疗及术后辅助化疗可以提高生存率,放疗疗效不肯定.预后仍然很差.中位生存时间为5-18个月, 2年的总生存率不到20%.本文就本病的组织学起源、影像学表现、临床表现、治疗原则及预后进行综述.     

足部骨肿瘤治疗结果的回顾性分析

目的 探讨足部骨肿瘤的发病特点,提高足部肿瘤的诊治水平.方法 回顾分析2000年3月-2009年4月收治的足踝部骨肿瘤32例,男18例,女14例.平均年龄36岁（13～61岁）.其中Ewing肉瘤2例,恶性纤维组织细胞瘤1例,软骨肉瘤3例,转移瘤1例,骨巨细胞瘤2例,软骨母细胞瘤3例,骨囊肿7例,骨样骨瘤2例,内生软骨瘤6例,骨软骨瘤1例,动脉瘤样骨囊肿4例.骨囊肿治疗方法采取透视下经皮注射生物蛋白胶及激素治疗;骨样骨瘤采用CT引导下经皮穿刺微波热疗术;其他良性肿瘤采用常规刮除植骨术.恶性肿瘤采用联合治疗方法,包括术前化放疗、术中磨钻及蒸馏水浸泡、术后放化疗等.结果 1例恶性纤维组织细胞肉瘤术后10个月复发行小腿截肢术,术后12个月死亡.2例Ewing肉瘤术后因转移死亡.感染1例,伤口换药8个月愈合.因异体骨排斥渗液伤口延迟愈合1例,术后9个月痊愈.余29例随访1-59个月（平均19.3个月）,长期无瘤生存.结论 随着微创概念的引进及新辅助化疗的不断进展,经皮注射生物蛋白胶和激素、经皮微波热疗术在足部骨肿瘤中的应用已经取得初步成果,创伤小,恢复快;各种物理治疗如磨钻、60°蒸馏水等的术中应用可降低复发率.     

Evidence-based chemotherapy for patients with bone and soft part sarcoma

In the present paper, we review the evidence for chemotherapy in patients with bone and soft part sarcoma and discuss the contributions and improvements made by chemotherapy to the treatment of patients with bone and soft part sarcoma. In the osteosarcoma and Ewing's sarcoma family, neoadjuvant and adjuvant chemotherapy have improved the 5-year disease-free survival to 60%, and limb-salvage operations have improved this to 70-80% in cases of non-metastatic malignant bone tumor. Several trials were conducted in order to overcome rate relapses and metastatic bone sarcoma. With osteosarcoma, thoracotomy improved the survival of lung metastatic patients, but CDDP-ADM branch switched according to the neoadjuvant chemotherapy and failed to elevate the continuous disease-free survival of patients. Dose intensive use of cytotoxic drugs with G-CSF or autologous bone marrow transplantation and multidrug programs were conducted in preliminary studies and achieved favorable results in a high risk factors group for tumors of the Ewing's sarcoma family. Surgical techniques have brought improvements in the treatment of soft tissue sarcoma, but there has been no impact by chemotherapy. Ifosfamide and adriamycin combination is being evaluated in the treatment of local advanced and metastatic soft part sarcoma by local control rate or survival from relapse.     

Chemotherapy in the management of osteosarcoma and Ewing's sarcoma

Sarcomas of bone are rare malignancies diagnosed in fewer than 3000 individuals yearly in the United States. Ewing's sarcoma and most osteosarcoma are high-grade neoplasms and account for approximately one half of bone sarcoma cases. Fewer than 20% of patients presenting with localized Ewing's sarcoma or osteosarcoma are cured with surgery alone. Current management typically involves collaboration among orthopedic oncologists, medical oncologists, musculoskeletal radiologists, sarcoma pathologists, and radiation oncologists. Modern multidisciplinary management of Ewing's sarcoma and osteosarcoma has improved the cure rate of patients with localized disease to more than 50%. Primary chemotherapy for high-grade bone sarcomas often involves intensive, multiagent regimens, and few secondary chemotherapy options are available to treat refractory or relapsed disease. Patient participation in clinical trials of novel therapies for Ewing's sarcoma and osteosarcoma should be strongly encouraged.     

Predictive factors of histological response to primary chemotherapy in Ewing's sarcoma

Clinicopathologic variables associated with a good histological response to primary chemotherapy in Ewing's sarcoma are identified. The histological response to preoperative chemotherapy in 243 cases of Ewing's sarcoma treated with neoadjuvant chemotherapy was analyzed in relation to different clinicopathological features (sex and age of the patients, tumor size, serum lactate dehydrogenase (LDH) levels, tumor site) and to the type and schedule of anticancer drugs delivered preoperatively according to three consecutive chemotherapy regimens. A higher rate of good responses was achieved with the use of ifosfamide and dactinomycin in addition to a conventional three-drug VAC regimen, suggesting that these drugs should be included from the beginning in neoadjuvant regimens for the treatment of Ewing's sarcoma. The analysis of event-free survival in 158 patients with a 4-year minimum follow-up confirmed that histological response to preoperative chemotherapy is a reliable predictor of outcome in Ewing's sarcoma.     

Predictive Factors of Histological Response to Primary Chemotherapy in Ewing's Sarcoma

Clinicopathologic variables associated with a good histological response to primary chemotherapy in Ewing's sarcoma are identified. The histological response to preoperative chemotherapy in 243 cases of Ewing's sarcoma treated with neoadjuvant chemotherapy was analyzed in relation to different clinicopathological features (sex and age of the patients, tumor size, serum lactate dehydrogenase (LDH) levels, tumor site) and to the type and schedule of anticancer drugs delivered preoperatively according to three consecutive chemotherapy regimens. A higher rate of good responses was achieved with the use of ifosfamide and dactinomycin in addition to a conventional three-drug VAC regimen, suggesting that these drugs should be included from the beginning in neoadjuvant regimens for the treatment of Ewing's sarcoma. The analysis of event-free survival in 158 patients with a 4-year minimum follow-up confirmed that histological response to preoperative chemotherapy is a reliable predictor of outcome in Ewing's sarcoma.     

High-dose chemotherapy with autologous peripheral blood stem cell transplantation (PBSCT) for refractory bone and soft tissue sarcomas]

High-dose chemotherapy with autologous peripheral blood stem cell transplantation was administered to 10 patients with refractory bone and soft tissue sarcoma (2 patients with primitive neuroectodermal tumor, 4 patients with Ewing's sarcoma, 3 patients with synovial sarcoma and one patient with osteosarcoma). Busulfan 4 mg/kg x 4, melphalan 140 mg/m2 and thiotepa 200 mg/m2 x 3 were used in the high-dose chemotherapy. Complications related to the treatment were limited to one patient who developed hepatic veno-occlusive disease, no serious complications were seen in the other patients. Four patients died of their disease, one patient was alive with the disease and 5 patients were alive with no evidence of disease. The prognosis for non-resectable primitive neuroectodermal tumor and Ewing's sarcoma is said to be very poor. However, there are some patients in whom the disease is kept in remission by high-dose chemotherapy with autologous peripheral blood stem cell transplantation, so this therapy may be a possible substitute for radical operation. With spindle cell sarcomas, the efficacy of this treatment was temporary, so it will be necessary to investigate frequent high-dose chemotherapy and to change the high-dose chemotherapy regimen.     

Extraskeletal Ewing's sarcoma

BACKGROUND: Ewing's sarcoma usually is identified as a primary malignancy of bone affecting children and young adults. Extraskeletal Ewing's sarcoma is rare, and very few data are available addressing optimal surgical and oncologic treatment modalities. METHODS: The authors chose to review retrospectively 24 patients with extraskeletal Ewing's sarcoma treated at the study institution with modern multimodality therapies. Anatomic location, tumor size, patient age at diagnosis, stage of disease at the time of diagnosis, surgical margins, radiation dose, and the type and dose of chemotherapy were documented for every patient. Follow-up averaged 64 months for surviving patients. RESULTS: The overall 5-year survival rate was 61% and the disease free survival rate was 54%. A multivariate analysis found that younger age at the time of diagnosis was associated with improved 5-year survival and disease free survival (P = 0.008 and P = 0.005, respectively). Patients who underwent wide resection and less-than-wide resection had better overall survival (P = 0.001 and P = 0.015, respectively) and disease free survival (P = 0.002 and P = 0.024) compared with those who underwent no attempt at surgical resection. Patients who underwent a wide resection had an improved overall survival compared with those who underwent a less-than-wide resection (P = 0.045). The size of the lesion (P = 0.277) and the presence of metastatic disease at the time of diagnosis (P = 0.219) were not found to be significant prognostic factors. CONCLUSIONS: Age and surgical treatment were found to be important prognostic variables in the treatment of extraskeletal Ewing's sarcoma. No other variables, such as tumor size, tumor location, stage of disease, or radiation therapy, were found to improve survival. Surgical resection should be considered for all patients with extraskeletal Ewing's sarcoma.     

根治性放疗同步化疗治疗局部晚期尤文氏肉瘤家族肿瘤的临床效果#br#

目的评价根治性放疗同步化疗治疗局部晚期尤文氏肉瘤家族肿瘤的临床效果。方法回顾性分析2012年6月至2015年12月新疆医科大学附属肿瘤医院收治的24例局部晚期尤文氏肉瘤家族肿瘤行根治性放疗同步化疗患者的临床资料,男15例,女9例;骨病变12例,骨外病变12例;腰椎3例,骶骨3例,小腿6例,上臂3例,肩胛骨3例,锁骨3例,腰大肌3例;24例均无法广泛切除。先行4周期化疗,方案：长春新碱+阿霉素+环磷酰胺/异环磷酰胺+依托泊苷（VAC/IE）交替方案各2周期,每3周重复1次,再行调强放疗,处方剂量：56 Gy/28f,2 Gy/次,5次/周,6周完成;放疗过程中原方案同步化疗2周期,再按原方案化疗10个周期;治疗过程中,每2周期化疗后根据实体肿瘤疗效评价标准（RECIST）评估原发病灶局部情况;治疗结束后,通过定期复查进行随访。结果同步放化疗后,全组24例患者中6例完全缓解（CR）,12例部分缓解（PR）,6例疾病稳定（SD）;总有效率（CR+PR+SD）为100%。5年无进展生存率（RFS）为58.3%,5年总生存率（OS）为62.5%。放化疗后发生Ⅰ～Ⅱ度骨髓抑制15例,Ⅲ～Ⅳ度骨髓抑制9例;Ⅰ～Ⅱ度胃肠道反应24例,1～2级急性放射性皮炎24例。结论根治性放疗同步化疗治疗局部晚期尤文氏肉瘤家族肿瘤的效果确切,耐受性良好,对于不能手术保肢或肿瘤不可切除的患者可选择该治疗方案。     

Effectiveness of Ecteinascidin-743 against drug-sensitive and -resistant bone tumor cells.

PURPOSE: The identification of new drugs is strongly needed for bone tumors.Ecteinascidin-743 (ET-743), a highly promising antitumor agent isolated from the marine tunicate Ecteinascidia turbinata, is currently under Phase II clinical investigation in Europe and the United States for treatment of soft tissue sarcoma. In this study, we analyzed the preclinical effectiveness of this drug in osteosarcoma and Ewing's sarcoma. EXPERIMENTAL DESIGN: The effects of ET-743 were evaluated against a panel of human osteosarcoma and Ewing's sarcoma cell lines characterized by different drug responsiveness and compared with the effects of standard anticancer agents. In addition, combination treatments with ET-743 and the other standard chemotherapy agents for sarcoma were analyzed to highlight the best drug-to-drug interaction RESULTS: A potent activity of ET-743 was clearly observed against both drug-sensitive and drug-resistant (multidrug-resistant, methotrexate- and cisplatin-resistant) bone tumor cells at concentrations that are easily achievable in patients (pM to nM range). Ewing's sarcoma cells appeared to be particularly sensitive to the effects of this drug. The analysis of the effects of ET-743 on cell cycle, apoptosis, and differentiation indicated that both osteosarcoma and Ewing's sarcoma cells had a slower progression through the different phases of the cell cycle after treatment with ET-743. However, the drug was able to induce a massive apoptosis in Ewing's sarcoma but not in osteosarcoma cells. In the latter neoplasm, ET-743 showed a differential effect, as indicated by the significant increase in the expression and activity of alkaline phosphatase, a marker of osteoblastic differentiation. Concurrent exposure of cells to ET-743 and other chemotherapeutic agents resulted in greater than additive interactions when doxorubicin and cisplatin were used, whereas subadditive effects were observed with methotrexate, vincristine, and actinomycin D. CONCLUSIONS: Overall, these results encourage the inclusion of this drug in the treatment of patients with bone tumors, although a careful design of new regimens is required to identify the best therapeutic conditions.     

Periosteal Ewing's sarcoma

Ewing's sarcoma is a small cell malignant tumor that usually arises in the medullary cavity of bone. Less frequently, it originates in soft tissue and may secondarily invade underlying bone. The origin of Ewing's sarcoma in a periosteal location without extension into either the bone or adjacent soft tissue has not been clearly documented. Other malignant tumors of bone (e.g., osteosarcoma) appear to have a somewhat better prognosis when confined between periosteum and bone. The case of a patient with a periosteal Ewing's sarcoma who received a radical excision and postoperative chemotherapy and who is without evidence of disease with over 2 years follow-up is reported.     

In vitro radiation studies on Ewing's sarcoma cell lines and human bone marrow: application to the clinical use of total body irradiation (TBI)

Patients with Ewing's sarcoma who present with a central axis or proximal extremity primary and/or with metastatic disease have a poor prognosis despite aggressive combination chemotherapy and local irradiation. In this high risk group of patients, total body irradiation (TBI) has been proposed as a systemic adjuvant. To aid in the design of a clinical TBI protocol, we have studied the in vitro radiation response of two established cell lines of Ewing's sarcoma and human bone marrow CFUc. The Ewing's lines showed a larger Do (1.26 Gy, 2.04 Gy) and n (6.0, 3.2) compared to the bone marrow CFUc (Do = 0.86 Gy, n = 1.2). No repair of potentially lethal radiation damage (PLDR) was found after 4.5 Gy in plateau phase Ewing's sarcoma cells. A theoretical split dose survival curve for both the Ewing's sarcoma lines and human bone marrow CFUc using this TBI schedule shows a significantly lower surviving fraction (10(-4)-10(-5] for the bone marrow CFUc. Based on these in vitro results, two 4.0 Gy fractions separated by 24 hours is proposed as the TBI regimen. Because of the potentially irreversible damage to bone marrow, autologous bone marrow transplantation following the TBI is felt to be necessary. The details of this clinical protocol in high risk Ewing's sarcoma patients are outlined.