联合化疗加放疗治愈一例复发性头部尤文氏肉瘤

联合化疗加放疗治愈一例复发性头部尤文氏肉瘤第一军医大学南方医院（５１０５１６）何杏，陈允清，彭林尤文氏肉瘤为青少年多发的恶性肿瘤之一，但发生在颅骨极为少见，约占０．６６％～６％［１］，且很少存活。我院１９８８年收治１例头部巨大尤文氏肉瘤，经手术、放疗...     

Ewing肉瘤诊断与治疗的体会（附9例报告）

Ewing肉瘤(尤文肉瘤)是骨科少见的一种高度恶性低分化的小圆细胞原发骨肿瘤。对青少年的危害极大,目前在诊断与治疗上存在不少问题,在治疗方法上有颇多争议。本文通过9例Ewing肉瘤的诊治提出了长管状骨及扁平骨Ewing肉瘤的特征及诊断方法。减少了误诊率;同时对其治疗提出术前全身化疗、术中局部化疗、术后化疗加放疗的综合治疗方案,以减少肿瘤复发及转移机率,为保存患肢功能,提高病人5年存活率,我们认为这是一种治疗Ew-ing氏肉瘤的有效方案。     

躯干中轴部位局限期尤文氏肉瘤家族肿瘤的综合治疗效果分析#br#

目的探讨躯干中轴部位尤文氏肉瘤家族肿瘤（ESFT）的临床特点及治疗策略。 方法从新疆医科大学附属肿瘤医院2008年1月至2017年5月收治的发生于躯干中轴部位的79例局限期ESFT患者中,筛选出有完整随访资料者67例;根据治疗方式分为综合治疗组39例（化疗+手术+放疗15例、化疗+手术12例及化疗+放疗12例）和单一治疗组28例（化疗10例、手术12例及放疗6例）,采用RECIST 11版标准评价近期疗效,根据随访资料分析远期生存情况。 结果综合治疗组的有效率为769％（30／39）,高于单一治疗组的500％（14／28）,差异有统计学意义（P＜005）;综合治疗组的中位总生存期（OS）和无事件生存期（EFS）分别为570个月和360个月,优于单一治疗组的190个月和80个月,差异有统计学意义（P＜005）。39例综合治疗者中27例接受手术+放疗+化疗或手术+放疗的有效率、中位OS和EFS分别为815％（22／27）、480个月和320个月,优于12例接受化疗+放疗的667％（8／12）、380个月和235个月,差异有统计学意义（P＜005）。39例综合治疗者中24例接受阿霉素联合异环磷酰胺（ADM+IFO）方案的有效率为625％（15／24）,与其余15例（非ADM+IFO方案）的733％（11／15）相比,差异无统计学意义（P＞005）;24例接受ADM+IFO方案的中位OS和EFS均＞640个月,与其余15例（非ADM+IFO方案）的580个月和340个月相比,差异亦无统计学意义（P＞005）。 结论综合治疗能改善发生在躯干中轴部位局限期ESFT患者的疗效和生存。化疗、手术联合放疗的治疗模式在疗效和生存方面优于化疗联合放疗模式。     

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     

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。     

56例软组织肉瘤的非计划性综合治疗分析

目的：评价放射治疗在软组织肉瘤治疗中的作用。方法：56例均为计划性的综合治疗，其中局部切除术后放疗27例，广泛切除术后放疗4例，剖腹探查术后放疗4例，局切后放疗加化疗5例，术后化疗6例。用^60Co常规分割照射，化疗用CYVADIC方案。结果：全组3、5、10年生存率分别为55．4％（31／56）、55．8％（24．43）、30．8％（8．26）。其中局切组5年生存率41．7％（10／24），广切组75．0（9／12）局切组放疗前术后复发率78．1％（25／32），放疗后复发率18．8％（6／32），差异有极显著性（P＜0．001）；广切组放疗后复发率14．4％（2／14）。结论：术后足量放疗可以提高软组织肉瘤保守手术后生存率，减少术后复发，尤其是广泛切除术后放疗，效果更明显。     

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 …     

原发性乳腺肉瘤40例临床分析

目的探讨原发于乳腺肉瘤的合理治疗模式。方法回顾性分析40例PBS患者的临床资料和治疗情况。结果40例患者均为女性,根据AJCC分期标准：Ⅰ期17例,Ⅱ期17例,Ⅲ期6例。其中叶状囊肉瘤25例,中位年龄40．4岁（21—70岁）,病理分级包括Ⅰ级6例、Ⅱ级12例、Ⅲ级7例,手术辅助化疗2例,单纯手术治疗23例（92．0％）,5年总生存率（OS）为85．1％。非叶状囊肉瘤15例,中位年龄36．1岁（15～55岁）,其中纤维肉瘤7例,横纹肌肉瘤、平滑肌肉瘤、癌肉瘤和脂肪肉瘤各2例,手术加化疗和（或）放疗5例,单纯手术治疗10例（66．7％）,5年OS为43．4％。结论原发于乳腺的叶状囊肉瘤单纯手术治疗疗效较好,而原发于乳腺的非叶状囊肉瘤单纯手术治疗则预后较差,须综合治疗才能改善预后。     

38例子宫内膜间质肉瘤的诊断和治疗

目的：研究子宫内膜间质肉瘤的诊断和治疗方法。方法：对３８例子宫内膜间质肉瘤进行回顾性分析,其中Ⅰ期１３例,Ⅱ期１４全,Ⅲ期７例,Ⅳ期４例,３８例均经手术治疗。１０例术后补充放疗,１１例补充化疗,７例补充放疗加化疗。结果：本组病例总的３年及５年生存率分别为５４．３％（１９／３５）及４５．５％（１５／３３）,Ⅰ期病例３年及５年生存率分别为７６．９％（１０／１３）及６１．５％（８／１３）,Ⅱ期为５０．０％（７／１４）及４６．２％（６／１３）。结论：子宫内膜间质肉瘤的预后和组织类型、临床分期、治疗方法密切有关,综合运用手术、放疗、化疗及孕激素治疗能减少阴道及盆腔复发,提高生存率。     

1260例肺小细胞未分化癌综合治疗结果分析

目的：探讨肺小细胞未分化癌（SCLC）综合治疗的疗效及影响预后的因素。方法：对1260例SCLC患者（局限期732例,广泛期500例,无分期28例）进行分组治疗,其中化疗＋放疗组553例,化疗＋放疗＋化疗组355例,放疗＋化疗组97例,单纯化疗组126例,单纯放疗组64例,手术＋化疗＋放疗组65例。局限期患者接受2－4个周期化疗,化疗方案有COMC、COMP、COMVP和CE－CAP;放疗40－70Gy/4-7周,照射野包括原发灶、同侧肺门、相应纵隔和双侧锁骨上。广泛期患者以化疗为主,姑息放疗。结果：全组CR率26．7％,PR率52．3％,总有效率79．0％。局部复发率58．8％,远处转移率61．5％。1,3,5年生存率分别为50．2％、14．7％和11．7％,中位生存期12个月。经单因素和多因素分析显示,治疗年代、性别、年龄、分期和治疗方法均有统计学意义（P＜0．05）。化疗＋放疗＋化疗组的疗效好于化疗＋放疗组,但差异无显著性。结论：对SCLC患者应争取早诊断早治疗,局限期患者应尽量争取以手术为主的综合治疗。早放疗稍好于晚放疗。     

Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan: the Japanese Musculoskeletal Oncology Group cooperative study

BACKGROUND: Ewing sarcoma family of tumors (ESFT) of bone is extremely rare in Japan. The objectives of the current study were to assess the clinical outcome and prognostic factors of patients with ESFT of bone in Japan and to compare them between Euro-American and Japanese populations. METHODS: The authors conducted a retrospective analysis of 243 patients who were treated for ESFT of bone in Japan between 1981 and 2003. Local therapy was surgery in 35% of patients, surgery combined with radiotherapy in 40% of patients, radiotherapy alone in 22% of patients, and no local treatment in 3% of patients. All but 3 patients received various regimens of multidrug chemotherapy. RESULTS: The median patient age was 16 years. The primary disease sites were the trunk in 53% of patients and the extremities in 47% of patients. Forty-one patients had metastases at presentation. The median follow-up was 66 months. A univariate survival analysis demonstrated that patients who had metastases at presentation, primary site in the trunk, age >or=16 years, tumor size >or=10 cm, tumor that responded poorly to induction chemotherapy, and local treatment with radiotherapy alone had a significantly worse event-free survival (EFS). A multivariate analysis further verified that the former 3 factors were significant adverse prognostic factors. Of 201 patients with localized disease, 45 patients who received current chemotherapy regimens that included ifosfamide and etoposide had a significantly better 5-year EFS rate (67.6%) compared with other patients. CONCLUSIONS: The clinical outcome of patients with localized ESFT of bone in Japan has improved markedly with the use of current chemotherapy regimens that include ifosfamide and etoposide and has become comparable to the outcomes observed in other major series of Euro-American patients. The prognostic factors are also almost identical.     

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。     

Construction and validation of nomograms for non-metastatic Ewing sarcoma: A prognostic factor analysis based on the SEER database

Ewing sarcoma is the second most common osseous disease in children and adolescents. It presents with a poor prognosis due to the high degree of malignancy and distant metastasis. In order to predict the disease prognosis and investigate a suitable therapeutic strategy for Ewing sarcoma, the present study aimed to describe the clinical characteristics, and to construct and validate nomograms for patients with non-metastatic Ewing sarcoma. A total of 627 cases of non-metastatic Ewing sarcoma were retrospectively collected from the Surveillance, Epidemiology, and End Results database between 2005 and 2014. Survival analysis and a machine learning model were used to identify independent prognostic variables and establish nomograms to estimate overall survival (OS) and cause-specific survival (CSS). The nomograms were bootstrap internally validated and externally validated using non-metastatic Ewing sarcoma cases from the First Affiliated Hospital of Zhengzhou University. The accuracy was also assessed by comparing with current American Joint Committee on Cancer (AJCC) staging systems. The total series consisted of 627 patients with non-metastatic Ewing sarcoma with a mean age of 20.14 years. Age, tumor extension, sex, International Classification of Diseases for Oncology, 3rd Edition histology, surgery and chemotherapy were identified as independent risk factors for OS and CSS. The aforementioned outcomes were incorporated to construct the nomograms, and the concordance indices (C-indices) for internal validation of OS and CSS prediction were 0.791 and 0.813, which were higher than those for AJCC sixth edition (OS, 0.531; CSS, 0.534) and seventh edition (OS, 0.547; CSS, 0.561), while the C-indices for external validation of OS and CSS prediction were 0.834 and 0.825, respectively. In conclusion, age, sex, tumor extension and surgery were independent prognostic factors for both OS and CSS. In addition, with regard to OS, the Ewing sarcoma subtype was a poor factor and chemotherapy was a favorable one. Nomograms based on reduced Cox models attained a satisfactory accuracy in predicting the survival of patients with non-metastatic Ewing sarcoma and could assist clinicians in evaluating survival more accurately.     

Prognostic factors for local and distant control in Ewing sarcoma family of tumors.

BACKGROUND: Advances in the treatment of Ewing sarcoma family of tumors (ESFT) are the result of improvements in systemic and local therapies. The individual contributions of each treatment component cannot be analyzed separately; improvements in local and systemic control can influence each other. PATIENTS AND METHODS: We reviewed the records of 220 patients treated on institutional protocols from 1979 to 2004. Factors predictive of local and distant recurrence were analyzed. RESULTS: The median age at diagnosis was 13.7 years. Ninety-five patients relapsed at a median of 1.6 years. The 5-year overall survival estimate was 63.5% +/- 3.5%. The estimated 5-year cumulative incidence (CI) of local failure was 25.1% +/- 3.0%. Local failure was associated with treatment era (P < 0.001), tumor size (P = 0.037) and type of local control (P = 0.021). Systemic treatment intensification improved local control. The estimated 5-year CI of distant recurrence was 22.5% +/- 2.9%. Patients with localized disease (P < 0.001), smaller tumors (P = 0.018) and those who received surgery +/- radiation for local control (P = 0.023) had lower CI of distant failure. CONCLUSIONS: Successful treatment of ESFT requires optimal systemic and local therapy. Both treatment modalities are intertwined and the control of both local and distant disease is the result of the combined approach.     

Survival of Patients with Ewing’s Sarcoma in Yazd-Iran

Background: The Ewing’s sarcoma family is a group of small round cell tumors which accounts for 10-15% of all primary bone neoplasms. The aim of this study was to evaluate the survival of Ewing’s sarcoma patients in our province and to determine of influencing factors. Materials and Methods: All patients with documented Ewing’s sarcoma/ primitive neuroectodermal tumor(PNET) family pathology were enrolled in this study during a period of eight years. For all of them local and systemic therapy were carried out. Overall and event free survivaland prognostic factors were evaluated. Results: Thirty two patients were enrolled in the study. The median age was 17.5 years. Twenty (65.2%) were male and 9 (28.1%) were aged 14 years or less. Mean disease free survival was 26.8 (95%CI; 13.8-39.9) months and five year disease free survival was 26%. Mean overall survival was 38.7 months (95%CI; 25.9-50.6) and median overall survival was 24 months. Five year overall survival was 25%. From the variables evaluated , only presence of metastatic disease at presentation (p value=0. 028) and completeresponse (p value =0. 006) had significant relations to overall survival. Conclusions: Survival of Ewing’s sarcoma in our province is disappointing. It seems to be mostly due to less effective treatment. Administration of adequatechemotherapy dosage, resection of tumor with negative margins and precise assessment of irradiation volume may prove helpful.     

Favorable outcome of Ewing sarcoma family tumors to multiagent intensive preoperative chemotherapy: a single institution experience

BACKGROUND: Aim of our study was to evaluate the efficacy of multiagent intensive preoperative chemotherapy in patients with Ewing sarcoma family tumors (ESFT), in order to succeed a better percentage of necrosis before surgical resection. PROCEDURE: Eighteen patients with ESFT were treated with the same multiagent intensive preoperative protocol. 5/18 patients had bone Ewings sarcoma (EWS) and 13/18 had peripheral primitive neuroectodermal tumor (PNET). None had metastases at diagnosis. Chemotherapy consisted of 5 or 6 cycles with vincristine, cisplatin, cyclophosphamide, and Adriamycin, followed by 12 cycles of vincristine, cyclophosphamide, and actinomycin-D. Five patients with EWS underwent total resection after 5-6 cycles of preoperative chemotherapy and prosthetic replacement was performed in two of them. In 3/13 patients with PNET the tumor was resected at diagnosis and in 1/13 after 5 cycles of chemotherapy, while 9/13 patients received chemotherapy only and/or radiotherapy. RESULTS: In patients with EWS, the histologic specimens of the resected tumors showed that tissue necrosis was 100% in four patients and 95% in one patient. The good histologic response reflects the effectiveness of this regimen in all ESFT. No patient had topical recurrence or developed metastatic disease during follow-up period (2-13 years, mean time 7.4 years). All patients had the scheduled cycles without delays or dose reductions. There were no major side effects of chemotherapy. CONCLUSIONS: The intensive chemotherapy schedule, comprising of 5-6 cycles preoperatively, seems to maximize the percentage of tumor necrosis, thus improving outcome. Our study implies that this combined therapy may improve the prognosis of ESFT.     

Ewing sarcoma

Ewing sarcoma is the second most frequent primary bone cancer affecting children or young adults. Advances in molecular biology have revealed common chromosomal translocations such as EWS-FLI 1 among Ewing sarcoma and related diseases such as primitive neuroectodermal tumor (PNET), so these are considered as Ewing sarcoma family tumor (ESFT). Although fewer than 10% of patients with ESFT survived before establishment of modern multiagent chemotherapy, the multimodal therapeutic regimens including combination chemotherapy, radiotherapy, and surgery can cure 60% of patients with localized disease, due to the collaborative research in European-American or the international trials. The standard chemotherapy for localized ESFT now comprises vincristine, actinomycin D, cyclophosphamide and doxorubicin (VACD) in Europe or vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC-IE) in North America. Meanwhile, those with metastatic disease have a much worse outcome with an approximately 10-30% 5-year event-free survival rate. New American-European collaborative trials such as EURO-E.W.I.N.G.99 are in progress for further improvement of the cure rate in localized and metastatic ESFT. In Japan, Japan Ewing Sarcoma Study Group (JESS) phase II clinical trial for localized ESFT, and some clinical trials including new drugs are ongoing and waiting for results.     

Adjuvant and neoadjuvant chemotherapy for Ewing sarcoma family tumors in patients aged between 40 and 60: report of 35 cases and comparison of results with 586 younger patients treated with the same protocols in the same years

BACKGROUND: The clinical and pathologic features of 46 patients 40 to 60 years old with Ewing sarcoma family tumor (ESFT) diagnosed at the authors' institute between 1972 and 2000 were reviewed. METHODS: Ten patients with metastatic tumors at presentation went elsewhere for treatment; 35 of 36 remaining cases with localized disease were treated at the authors' institution according to different chemotherapy protocols activated in successive years. In patients with nonmetastatic tumors local treatment was surgery in 9 patients, radiotherapy in 16, and surgery followed by radiotherapy in 10. RESULTS: At follow-up times ranging from 6 and 34 years (mean, 17.8 years), 15 patients (42.9%) remained continuously disease-free, 19 experienced recurrence, and 1 died of chemotherapy-related toxicity. The 5- and 10-year event-free survivals were 42.9% and 35.2%, respectively, and the 5- and 10-year overall survivals were 46.1% and 42.8%, respectively. Comparing this group of patients with 586 cases of younger patients seen in the same period at Rizzoli, the only difference between the 2 groups was a significantly higher rate of tumors located in the soft tissues with a larger volume in the older group. The results achieved were comparable in the 2 groups, although the older group had a lower chemotherapy dose-intensity and a higher rate of WHO grade 4 hematologic toxicity. CONCLUSIONS: For patients with localized disease treated with adjuvant and neoadjuvant chemotherapy the results were essentially comparable in the 2 groups. It is concluded that patients 40 years or older with ESFT should be treated in the same way as younger patients and included in treatment trials for these tumors.     

Chemotherapy response is an important predictor of local recurrence in Ewing sarcoma

BACKGROUND: Local recurrence in Ewing sarcoma is associated with a poor prognosis. The purpose of the study was to determine the factors that predict local recurrence after surgical treatment of the primary tumor. METHODS: Between 1990 and 2001, 64 patients underwent surgical resection of Ewing sarcoma. Surgical margins were assessed histologically and radiologically. Response to preoperative chemotherapy was determined by detailed specimen mapping. Local recurrence-free survival (LRFS) was calculated by Kaplan-Meier analysis. Multivariate analysis was performed with the Cox proportional hazards model. RESULTS: A number of factors were found to be associated with local recurrence on univariate analysis. Patients with a good response to chemotherapy (> or = 90% tumor necrosis), had superior LRFS at 5 years (86% vs 51%, P = .015). Central site of disease was associated with an increased rate of recurrence. The LRFS at 5 years was 50% for the chest wall, 74% for pelvic/scapular, and 86% for extremity tumors (P = .083). Positive surgical margin was not a strong predictor of recurrence (P = .72). A critical analysis of minimal surgical margin based on preoperative magnetic resonance imaging (MRI) and computed tomography (CT) scans also failed to reveal an association between margin and local recurrence. In multivariate analysis, the 2 independent predictors of local recurrence were histological response to chemotherapy and central site of disease. CONCLUSION: Local recurrence after surgical resection is a complex phenomenon. An important predictive factor is the response to chemotherapy. In the current study, this seems to have the largest impact. Central site of disease may be a second independent predictive factor.