外伤后眼眶横纹肌肉瘤4例

外伤后眼眶横纹肌肉瘤４例北京武警总医院眼科侯世科，张秀芬，唐东润（北京１０００３９）眼眶原发性横纹肌肉瘤（Ｒｈａｂｄｏｍｙｏｓａｒｃｏｍａ，以下简称ＲＭＳ）是儿童时期较常见的原发恶性肿瘤，成年人较少见。其发病原因不明。我们于１９８６～１９９２年，收治...     

眼眶横纹肌肉瘤30例MRI表现

横纹肌肉瘤（rhabdomyosarcoma）是儿童时期最常见的一种软组织肉瘤,但发生于眼眶者少见[1],影像表现报道则更少[2-5].为了加深影像科、眼科医师对本病的认识及提高术前诊断准确率,笔者将本院2008-05至2013-09收集的眼眶横纹肌肉瘤30例磁共振成像（magnetic resonance imaging, MRI）表现做一总结分析.     

MRI对眼眶横纹肌肉瘤的诊断价值

目的 探讨MRI对眼眶横纹肌肉瘤(rhabdomyosarcoma,RMS)的诊断价值.资料与方法 回顾性分析28例经手术病理证实的眼眶RMS患者的MRI表现.结果 28例中胚胎型21例,未作病理分型7例.均行MRI.病变位于肌锥内间隙23例,肌锥外间隙5例.11例原发,17例术后复发.肿瘤形态多较规则,边界尚清.21例病变较大,挤压视神经及眼球,造成视神经及眼球移位.T1WI上24例较眼外肌呈中等偏低信号,4例呈稍高信号.T2WI上信号强度略高于眼外肌,26例低于眶内脂肪.脂肪抑制序列上呈高信号.结论 眼眶RMS在MRI上有一定的信号特点,而且MRI可以清楚地显示病变范围及其与眶内周围组织的关系,对于眼眶RMS的诊断有重要意义.     

成人眼眶腺泡状横纹肌肉瘤一例

成人眼眶腺泡状横纹肌肉瘤一例汪洁许平患者女，４１岁。因左眼肿物１月，剧痛３天入院。体检：左上下睑高度肿胀，眶内上方触及质硬肿块，边界不清，触痛（），球结膜充血（），高度水肿，脱出睑裂外，虹膜纹理青棕色，瞳孔４ｍｍ，对光反射消失，晶体透明，玻璃体稍...     

儿童眼眶横纹肌肉瘤的治疗(附21例报告)

横纹肌肉瘤是常见的儿童软组织恶性肿瘤。眼眶横纹肌肉瘤占全身横纹肌肉瘤的10%。手术、放化疗联合合理应用可以明显改善眼眶横纹肌肉瘤的预后。本文分析武警总医院采用放射治疗眼眶横纹肌肉瘤的资料,旨在为本病的治疗提供经验。     

眼眶横纹肌肉瘤的临床特点及影像学评价

目的 探讨眼眶横纹肌肉瘤的临床特点及影像学表现。材料与方法 结合16例经病理证实的眼眶横纹肌肉瘤的临床特点,回顾性分析其B超、CT和MRI特征。结果 眼眶横纹肌肉瘤主要临床特点为发展迅速的进行性眼球突出,眼眶内上象限为其好发部位。B超检查显示肿瘤为低或无回声区。16例CT平扫,14例与眼外肌呈均匀的等密度,边缘清楚。10例行增强扫描,明显强化4例,中等强化5例。骨质破坏5例。9例行MRI检查,T1WI肿瘤与眼外肌呈等信号或略低信号,T2WI呈高信号;6例行增强扫描,4例中等度强化,2例明显强化。在脂肪抑制和增强T2WI上呈高信号。结论 超声、CT和MRI,尤其CT和MRI能较准确的显示横纹肌肉瘤的部位、形态和内部结构,结构临床特征,能较准确作出定性诊断,对临床选择治疗方案有重要价值。     

眼眶胚胎型横纹肌肉瘤伴全身多处转移确诊延迟1例

1病例报告患者男，9岁。因左眼球突出2个月，加重10天入院。查体：神志清楚，被动半卧位。满月脸，左侧头顶部一圆形包块，12cm×15cm大小，质硬，深压痛，边界清，活动度差。双耳听力受损，左耳后见一2cm×3cm大小丘状隆起包块。左下颌颊侧及舌侧分别见5cm×4cm、6cm×7cm类圆形肿物。L4～5见一12cm×18cm大小局限性隆起，皮肤颜色正常，压痛（＋）。     

左眼眶横纹肌肉瘤1例

1 病历简介 患者，女，2岁。无诱因发现左眼上睑下垂1个月余，于2007年3月21日入院。无眼红，眼痛，发热，畏光，流泪等症状。左眼上睑下垂呈逐渐加重趋势。个人史、家族史、既往史无特殊。体查：生命征正常，神清，心、肺、腹未见异常。专科检查：双眼视力未配合检查。左眼上睑轻度肿胀、下垂，遮盖瞳孔约2／3，眼球轻度向下向前突出，未触及肿物，角膜透明，瞳孔较有侧大，直径约4mm，直接对光反射迟钝，眼球运动正常，眼底检查未合作。右眼未见异常。     

原发性肩背部横纹肌肉瘤1例

1 病历简介 患者,男,7岁,右肩背部肿块伴局部疼痛1月余.1月前右肩背部有蚕豆大小结节,最近明显增大,并在该肿块上长出了两个小肿块.查体:右肩背部有一约12cm×6cm肿块,边界清,质硬,活动度差,有压痛,无血管搏动,肿块上方有一约2.0cm×1.0cm和6.0×3.0cm 肿块.压痛明显,活动差.右侧腋窝可触及一约花生米大小结节,有压痛.     

眼眶粒细胞肉瘤1例

1病例报告 患者男,7岁。因右眼球突出,轻度肿胀伴视力下降7个月收治入院。眼科检查：右眼眼球突出,球结膜充血,水肿;眼球各方向活动受限;     

Radiotherapy in the treatment of orbital rhabdomyosarcoma]

The authors present the results obtained in 14 children affected with orbital rhabdomyosarcoma, who were studied between 1979 and 1991 at the I Dept. of Radiology and Radiotherapy of the II Policlinico, Naples, Italy. The patients (age range: 14 months to 15 years) had received radiotherapy and chemotherapy: the dose was 6000 cGy to the orbital region (one case was given 4500 cGy and then interrupted radiotherapy for personal reasons). Twelve children underwent a standard protocol, that is biopsy followed by immediate irradiation and chemotherapy; surgery was performed in 2 cases. 90% survival at 2 years was obtained in our series. All 14 patients are alive and free of detectable disease, after a minimum of 7 to a maximum of 126 months from diagnosis. In 3 children a locoregional relapse was observed 12, 15, and 16 months after clinical diagnosis. They have received a new cycle of radiation therapy and are now free of detectable disease. In all children, ocular structures have been spared, and the complications observed so far have been few. Therefore, the authors suggest that the combination of immediate radiotherapy and chemotherapy might represent an optimal tool for the treatment of orbital rhabdomyosarcomas.     

Imaging of pelvic rhabdomyosarcoma by bone scintigraphy

Urinary tract distortion caused by pelvic rhabdomyosarcoma was imaged during ^99mTc-oxidronate bone scintigraphy for suspected metastatic disease. This appearance correlated well with the anatomy defined on other imaging modalities. Bone scanning also proved valuable in detecting an otherwise occult osseous metastasis from this unusual tumor.     

Ethical Analysis as a Tool for Addressing Treatment Controversies: Radiotherapy Timing in Children With Orbital Rhabdomyosarcoma as a Case Example

PurposeThe treatment of orbital rhabdomyosarcoma is a topic of debate between North American and European clinicians, with the utility of radiation therapy as part of initial management in question. Despite differences in philosophy, the dominant North American approach of upfront radiation and the dominant European approach of radiation only in the event of recurrence yield a similar rate of overall survival. We sought to identify the ethical arguments for each approach.MethodsEstablished moral principles and appeals in contemporary medical ethics were utilized to identify the ethical arguments supporting each treatment approach. The potential for technologic advances to alter the analysis was considered.ResultsEmphasizing the principle of beneficence, the North American approach seeks to reduce recurrence rates. In contrast, the European approach seeks to avoid radiation-induced sequelae, emphasizing the principle of nonmaleficence. Both approaches are based on well-established ethical principles, evidence, and clinical experience. Thus, both approaches currently appear to have legitimacy and should be included in the informed consent process. However, if treatment-related toxicity is reduced through improvements in radiation delivery, the North American approach could emerge as ethically superior.ConclusionsEthical analysis can aid in addressing challenges that arise when professional practices and perspectives differ in the management of cancer patients.     

耳部原发性横纹肌肉瘤的临床与病理特征

目的 探讨耳部原发性横纹肌肉瘤的临床及病理特征及其与婴儿横纹肌纤维肉瘤、原始神经外胚瘤、恶性横纹肌样瘤等肿瘤的鉴别诊断。方法 用光学显微镜和免疫组织化学以及肉眼观察8例耳部原发性横纹肌肉瘤的临床病理学特征。结果 8例中男性7例,女性1例,平均年龄7．4岁,其中胚胎性横纹肌肉瘤7例,葡萄型横纹肌肉瘤1例。免疫组织化学检查阳性率分别为：Vimentin及Myoglobin100％,Desmin75％,Actin82％。结论 耳部原发性横纹肌肉瘤和其他部位的软组织横纹肌肉瘤相比,组织学上并无特殊,但临床及病理形态学诊断却较困难,常需免疫组织化学标记帮助方能确诊。     

儿童横纹肌肉瘤的回顾性分析

 目的 探讨儿童横纹肌肉瘤(rhabdomyosarcoma, RMS)的临床特点、综合治疗方法、疗效和预后。方法 回顾性分析武警总医院2003-01至2013-06初治的20例RMS患儿的临床资料,总结儿童RMS的临床特点、综合治疗方法、疗效及预后。结果 20例患儿全部获随访,中位随访时间为38个月;男女比例为2.3∶1;平均发病年龄4.25岁。所有患儿均经病理确诊,依据美国RMS研究组(IRS) 的分期标准,Ⅱ期6例,Ⅲ期9例,Ⅳ期5例;依据世界卫生组织( WHO) 分类,胚胎型17例,腺泡型2例,多形型1例;低危组0例,中危组15例,高危组5例。原发于头颈部18例,四肢1例,睾丸1例。结合上海儿童医学中心RMS 2002方案与美国(IRS)方案制定的方案,7例接受手术+化疗,13例接受以手术、化疗及放疗为主的综合规范治疗,所有患儿治疗后疗效评价:CR12例,PR2例,PD6例,CR率60%。5例高危患儿死亡,其中规范治疗的3例患儿平均随访时间为39.3个月,另2例平均随访时间为14.4个月;13例复发患儿在随访12个月时复发最多,其中7例接受手术联合化疗治疗。结论 胚胎型为儿童RMS多发的病理分型,临床表现缺乏特异性,手术、化疗联合放疗为主的综合规范治疗方法对提高RMS的预后有一定的作用。     

儿童横纹肌肉瘤的MRI表现及ADC值的诊断价值

目的 探讨儿童横纹肌肉瘤(RMS)的MRI特征表现及表观扩散系数(ADC)值的诊断意义.方法 回顾性分析27例经病理证实的儿童RMS的临床、病理及影像学资料.结果 27例RMS患者中,单发25例,多发2例,共29个病灶.病灶T2 WI信号多不均匀,T1WI呈低或稍低信号的占96％(26/27),T2WI呈高信号的占93％(25/27).所有病灶扩散加权成像(DWI)高b值扩散均受限,ADC值为(0.83±0.37)×10-3mm2/s.增强扫描明显强化的占100％,69％(9/13)不均匀强化,时间-信号曲线呈流入型.结论 儿童RMS的MRI表现有一定特征,平扫表现为稍长T1长T2信号,增强扫描明显强化,时间-信号曲线呈流入型,ADC值低于一般恶性肿瘤.     

Radiotherapy of orbital pseudotumor.

Recent advances in ultrasound and computed tomography have permitted localization of orbital lesions, and sophisticated high-energy irradiation techniques facilitate improved dose distribution without significant damage to normal tissue. Three patients treated for orbital pseudotumor are discussed.     

放疗加紫杉醇治疗胃癌的临床观察

目的探讨常规放疗加紫杉醇治疗胃癌的临床效果及毒副反应。方法回顾性分析我院2005年2月—2006年11月进行常规放疗加每周紫杉醇治疗的胃癌患者35例,放射治疗每周5次,每次1.8 Gy,总剂量43.2 Gy,对于残存肿瘤加量至50.4 Gy。紫杉醇每周1次,40 mg/m2,持续静滴3 h。结果35例按计划完成了治疗,总有效率为80%,1年总生存率为74%。放射性食管炎及胃炎是最常见的毒性反应,Ⅲ级6人(17%),Ⅳ级5人(14%)。结论常规放疗加紫杉醇治疗胃癌是有效的治疗方法,局部控制率好,毒副反应能够耐受,但远处转移的控制有待更进一步的研究。