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。     

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。     

右颊部Ewing肉瘤/原始神经外胚叶肿瘤PNET1例

1病例报告患者,男,21岁,于两年前感右颊部有异物感,自行触摸有一肿物,无明显疼痛不适,肿物逐渐长大,曾于半年前切除部分肿物,近两月来肿物生长迅速,遂住院治疗。患者既往体健,无外伤史。查体：患者颈胸腹部未见异常,浅表淋巴结无肿大。     

右颊部Ewing肉瘤/原始神经外胚叶肿瘤PNET1例

1 病例报告 患者,男,21岁,于两年前感右颊部有异物感,自行触摸有一肿物,无明显疼痛不适,肿物逐渐长大,曾于半年前切除部分肿物,近两月来肿物生长迅速,遂住院治疗.患者既往体健,无外伤史.     

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 …     

头颈部原始神经外胚叶瘤三例

1病例简介和治疗方法①病例1,患者男性,17岁。因右鼻外侧肿胀3 d,曾于外院就诊。体检:鼻面部外观明显隆起,偏右为著,区域约9 cm×7 cm,局部有压痛,双鼻腔狭小,未见新生物,未扪及肿大淋巴结。CT:右鼻前庭,双齿槽、前鼻嵴骨质不规则吸收破坏,伴软组织增生,边界不清,涉及皮下。经唇龈手术活检,病理诊断:(唇龈)原始神经外胚叶瘤。免疫组化:V im(+),CD99(+),NSE(+),Syn(-),LCH(-),CgA(-),Des(-)。予DDP、VCR、THP化疗六个疗程,局部放疗60 Gy。②病例2,患者女性,4岁。因右眼突出、痛一月左右,伴反复发热史,2004年8月来我院就诊。体检:右…     

外周原始神经外胚叶肿瘤23例临床分析

目的:探讨外周原始神经外胚叶肿瘤(pPNET)的临床特征,以指导诊疗和评估预后。方法:收集我院23例pPNET患者的临床资料进行回顾总结,分析其临床表现、诊断和治疗的方法,并随访其生存期。结果:男性13例,女性10例,平均年龄为27.3岁,好发于躯干、四肢和中轴软组织包括胸壁、脊柱旁和腹腔等,多以出现进行性增大的局部包块起病。实验室和影像学检查无特异性指标,依据病理学明确诊断,治疗以手术为主,辅以全身化疗及局部放疗。平均生存期为15.9个月,年龄<16岁、原发于腹腔、不规范治疗的患者预后差。结论:pPNET是一种多发于青少年的高度恶性肿瘤,依靠病理学诊断,尽早发现,及时手术,辅以放化疗可延长生存期。     

骨外成釉细胞瘤样变异型尤文肉瘤／原始神经外胚叶肿瘤的临床病理特征

目的：探讨骨外成釉细胞瘤样变异型尤文肉瘤（ AES）／原始神经外胚叶肿瘤（ PNET）的临床病理特征、诊断及鉴别诊断。方法对1例发生在头皮下的AES／PNET进行HE、免疫组化染色及分子遗传学检测,并结合文献进行复习。结果大体观察肿物边界尚清,无包膜,切面灰红色、质软。镜下：肿瘤细胞被多少不等、宽窄不一的纤维结缔组织分隔,呈大小不等的巢状、条索状、小叶状、腺样、等结构排列。其中肿瘤细胞巢呈上皮样生长方式是其最具特征的形态学表现,部分细胞巢的外周细胞核呈明显栅栏状排列。瘤细胞形态多样,体积小、核深染、胞质少的“暗细胞”,与周围体积大、染色质空泡状、胞质相对丰富的“亮细胞”混杂相间,并可见梭形细胞样或鳞状上皮样瘤细胞。免疫表型：肿瘤细胞CD99、FLI1、CKpan、低分子量CK弥漫阳性,Vimentin、Villin、高分子量CK、CD56、EMA局灶阳性,Ki-67增殖指数为85％;特殊染色PAS（＋）／D-PAS（－）,证实胞质内含丰富的糖原。分子遗传学：EWS-FLI1融合基因断裂。结论 AES是1种罕见的尤文肉瘤（ Ewing＇s Sarcoma,ES）／PNET变异亚型,需与多种小圆细胞肿瘤进行鉴别,预后很差。     

肺原发尤文肉瘤/原始神经外胚层瘤1例

尤文肉瘤/原始神经外胚层瘤是一种神经外胚层分化的小圆细胞恶性肿瘤。现报道1例肺原发尤文肉瘤/原始神经外胚层瘤患者的诊疗情况, 复习相关文献并进行讨论, 以提高对该类疾病的认识。     

80例外周原始神经外胚叶肿瘤的临床分析

目的 探讨外周原始神经外胚叶肿瘤（pPNET）的临床特点、诊断要点、治疗疗效及预后相关因素。方法 收集本院2001年3月至2015年7月收治的80例pPNET患者资料,回顾性分析其临床表现、诊断、治疗方法并随访其预后。结果80例pPNET患者中男性44例,女性36例,中位年龄22.0岁;原发于头颈部8例、四肢40例、椎体2例、椎管内7例、锁骨2例、胸壁5例、肺部4例、纵隔3例、腹盆腔8例、肛周1例,多以进行性增大的局部包块起病。实验室和影像学检查无特异性指标,均通过病理学检查明确诊断。41例（包括13例新辅助化疗）行完整切除术,39例仅行部分切除或活检术,所有患者均行化疗或放疗。截至2017年6月,全组死亡56例,中位总生存期为26个月,1、2、3、5年生存率分别为85.2%、54.3%、42.6%和28.5%。原发部位、肿块大小、有无远处转移、是否行完整切除与pPNET患者的总生存期有关,Cox多因素分析提示肿块大小和完整切除与否可作为判断pPNET患者预后的独立因素。结论pPNET是一类多发于青少年的高度恶性肿瘤,侵袭性强,易转移和复发,依靠病理学诊断,及早发现、手术及综合治疗对改善预后有重要意义。     

Ewing sarcoma outcomes in a country with limited resources: Egypt as an example

As the scarcity of published research that comprehensively and meticulously analyzed the patient, disease, and treatment factors of prognostic significance in Ewing sarcoma (EWS) in Egypt; This study aimed at assessing survival outcomes of EWS in Upper Egypt, delineating factors of prognostic significance in comparison to other leading oncology centers in Egypt and internationally. By retrospectively reviewing medical records of 85 patients with a verified diagnosis of EWS in the period from 2001 to 2015 at Pediatric and Medical Oncology Departments at South Egypt Cancer Institute; We gathered data relevant to the patient, disease, and treatment variables of the study. Survival was estimated using the Kaplan Meier method and differences between various groups were determined by log rank test. Univariable and multivariable analyses were performed using Cox regression. With a median follow-up period of 62.7 months (95% CI 52.2-73.2, SE=5.4) for the study patients, the estimates of event-free survival (EFS) and overall survival (OS) at 3 and 5 years were 42.1% and 50.6%, and 40.8% and 48.5%, respectively. Metastatic disease at initial presentation (HR=8.91, 95% CI, 4.00-19.9; P<0.0001) stood as the most powerful predictor of OS in the multivariable analysis, followed by surgery used as a local modality (HR=0.16, 95% CI, 0.06-0.44; P=0.0004). Response to neoadjuvant chemotherapy (HR=2.61, 95% CI, 1.11-6.13; P=0.028), primary tumor size (HR=2.49, 95% CI, 1.03-6.03; P=0.044) were also shown to be significantly associated with OS. Radiotherapy as a local modality, whose effect, apparently shown to increase the hazard of events occurrence in the univariable analysis, an effect that was reversed to reveal EFS advantage (HR=0.41, 95% CI, 0.18-0.95; P=0.036) after control of other variables. With 5-year OS of 48.5%, our survival results were comparable to those previously published from Egypt; however, differences still exist between centers due to varied representative study samples. However, outcomes in Egypt in general are still inferior to internationally published studies.     

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 sarcoma/primitive neuroectodermal tumor of the ureter:A case report and literature review

Ewing sarcoma/primary neuroectodermal tumors are rare,invasive,and small round blue cell tumors.There are few reports of its occurrence in the urinary system.Here,we present the first middle-aged female patient whose Ewing sarcoma primary site was in the ureter.The main clinical manifestation was intermittent hematuria.She was in good health after complete surgical resection and adjuvant radiotherapy.To date,there has been no recurrence or metastasis.Accurate early diagnosis and appropriate treatment can help prolong survival.18F-fluorodeoxyglucose positron emission tomography/computed tomography is expected to be an effective means of evaluating treatment effects and detecting metastasis and recurrence.In this article,besides introducing a case of Ewing sarcoma/primitive neuroectodermal tumor of the ureter,we review the literature to discuss the current status of diagnosis and treatment.     

Second cancers in patients with the Ewing sarcoma family of tumours

BackgroundPatients are at risk of second malignancies (SM) after treatment for Ewing sarcoma family of tumours (ESFT).MethodsWe performed a retrospective review of 237 patients with ESFT treated at our institution from September 1979 through to February 2004. Cumulative incidence (CI) of SM by the type of malignancy and treatment was estimated.ResultsTwelve patients with SM were identified. Secondary leukaemia (SL) developed in 8 patients (2 ALL, 6 MDS/AML), a median 2.6 years (range 1.4–19.6 years) after diagnosis of ESFT. Four patients had secondary solid tumours, a median 8.0 years (range 7.4–9.4 years) after the ESFT diagnosis. Five- and 10-year estimates of the CI of SM were 3.0 ± 1.1% and 4.7 ± 1.5%, respectively. Patients treated on recent protocols with higher cumulative doses or an increased dose intensity of alkylators and epipodophyllotoxins and the use of G-CSF had a higher estimated CI of SL than those in earlier studies (5-year CI 6.4 ± 2.4% versus 0.0 ± 0.0%, respectively, P = 0.004).ConclusionsPatients with ESFT are at risk for SM after treatment. The cumulative incidence of SM is higher with the current treatment protocols and may be related to the intensification of chemotherapeutic agents.     

High-dose chemotherapy and stem cell rescue for high-risk Ewing’s family of tumors

The prognosis for high-risk Ewing’s tumors has been improved by multimodal radiation and chemotherapy. Ewing’s family of tumors requires risk-adapted treatment. Risk stratification is dependent on stage, tumor localization and volume, and the pattern of disease spread at the time of diagnosis and the time of relapse. The concepts for high-dose therapy followed by hematopoietic cell transplantation in Ewing’s family of tumors are based on dose–response and dose–intensity relationships. This article will discuss the use of high-dose therapy followed by hematopoietic cell transplantation, focusing on recent progress with respect to agent combinations, dose and outcomes of therapy.     

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.