25例腹内型侵袭性纤维瘤病临床分析

[目的]探讨腹内型侵袭性纤维瘤病的临床特点、治疗方法和预后。[方法]回顾性分析1999年1月至2013年3月收治的25例腹内型侵袭性纤维瘤病患者的临床资料,生存分析采用Kaplan-Meier法。[结果]5、10年生存率分别为94.4%和70.7%,无复发生存率分别为68.7%和58.9%。肿瘤最大径≥10cm（χ2=5.074,P=0.024）和接受姑息性手术（χ2=6.131,P=0.013）的患者预后较差。[结论]腹内型侵袭性纤维瘤病术前难以确诊,首选手术治疗,肿瘤大小和能否根治性切除是其预后影响因素。     

侵袭性纤维瘤病诊治进展

侵袭性纤维瘤病不同于普通良性软组织肿瘤，表现为局部浸润生长．但未见转移。侵袭性纤维瘤病手术切除后，具有高局部复发率。放疗提高局部控制率，化疗应用于不宜手术和放疗患者是有益的。全文就侵袭性纤维瘤病的诊治作一综述。     

142例侵袭性纤维瘤病的临床特点及预后分析

目的 分析侵袭性纤维瘤病的临床特征及预后因素,为临床治疗提供依据.方法 回顾分析本院1983-2009年治疗的142例侵袭性纤维瘤病患者的临床资料,观察临床特点及治疗方式对预后影响.采用Logrank单因素分析及Cox多因素回归分析评估可能影响局部预后的危险因素.结果 随访率为93.7%,随访满5、10者分别为63例、6例.本组病例男女比例为1∶1.8,18～35岁女性为高发人群(25.4%).病变部位发生于躯干(55.6%)及四肢(31.7%)多见.5、10年局部复发率分别为24.4%、31.1%,生存率均为99.3%.单因素分析发现肿瘤大小(χ2=4.37,P=0.037)和切缘情况(χ2=12.36,P=0.002)为肿瘤复发的危险因素.多因素分析发现切缘情况为独立的复发危险因素(RR=2.129;χ2=9.47,P=0.002),放疗为侵袭性纤维瘤病的保护因素(RR=0.360;χ2=4.95,P=0.026).放疗后切缘阳性患者10年局部复发率从70.1%降至20.7%(χ2=4.22,P=0.040);而切缘阴性患者从19.8%降至10.4%(χ2=0.90,P=0.344).结论 根治性切除为侵袭性纤维瘤病的首选治疗,术后放疗可以降低切缘阳性患者的局部复发率,但对切缘阴性患者的意义尚需大样本临床研究证实.

Abstract：

Objective Aggressive fibromatosis is a rare kind of soft tissue tumor and was evaluated by few large studies. This study was to evaluate the clinical characteristics and identify the prognostic factors of this disease. Methods One hundred and forty-two patients with aggressive fibromatosis treated from January 1983 to August 2009 in Tianjin Medical University Cancer Hospital were retrospectively reviewed.The prognostic value of clinical and treatment factors was analyzed. Univariate analysis was performed with Log-rank test and Multivariate analysis was performed with Cox regression model. Results The follow-up rate is 93.7% and the median follow up time was 54 months (range,6 -208 months). Sixty-three patients had a minimum follow up time of 5 years and 6 patients had a minimum follow up time of 10 years. The male/female ratio was 1/1.84. The disease was most popular in women aged from 18 to 35 years old. The disease frequently occurred in the trunk (55.6%) and extremity (31.7%). All patients received surgery,and 46 received radiotherapy. The 5-year and 10-year local recurrence rates were 24. 4% and 31.1%,respectively. The 5-year and 10-year overall survival rates were both 99. 3%. Univariate analysis revealed that factors correlated with local recurrence were tumor size ( χ2 = 4. 37, P = 0. 037 ) and margin status (χ2 = 12. 36,P =0. 002). Multivariate analysis revealed that margin status was an independent risk factor (RR = 2. 219; χ2 = 9. 47,P = 0. 002) and radiotherapy was an independent protective factor ( RR = 0. 360;χ2 = 4. 95, P = 0. 026 ) for disease recurrence. When radiotherapy was delivered, the 10-year local recurrence rate decreased from 70. 1% to 20. 7% in patients with positive margin ( χ2 = 4. 22, P = 0. 040 )and decreased from 19.8% to 10.4% (χ2= 0.90, P= 0.344) in patients with negative margin.Conclusions Radical resection is the mainstay of treatment for aggressive fibromatosis. Postoperative radiotherapy can reduce the recurrent rate for patients with positive margin. In patients with negative margin,the role of radiotherapy should to be further evaluated in large clinical trials.     

142例侵袭性纤维瘤病的临床特点及预后分析

Objective Aggressive fibromatosis is a rare kind of soft tissue tumor and was evaluated by few large studies. This study was to evaluate the clinical characteristics and identify the prognostic factors of this disease. Methods One hundred and forty-two patients with aggressive fibromatosis treated from January 1983 to August 2009 in Tianjin Medical University Cancer Hospital were retrospectively reviewed.The prognostic value of clinical and treatment factors was analyzed. Univariate analysis was performed with Log-rank test and Multivariate analysis was performed with Cox regression model. Results The follow-up rate is 93.7% and the median follow up time was 54 months (range,6 -208 months). Sixty-three patients had a minimum follow up time of 5 years and 6 patients had a minimum follow up time of 10 years. The male/female ratio was 1/1.84. The disease was most popular in women aged from 18 to 35 years old. The disease frequently occurred in the trunk (55.6%) and extremity (31.7%). All patients received surgery,and 46 received radiotherapy. The 5-year and 10-year local recurrence rates were 24. 4% and 31.1%,respectively. The 5-year and 10-year overall survival rates were both 99. 3%. Univariate analysis revealed that factors correlated with local recurrence were tumor size ( χ2 = 4. 37, P = 0. 037 ) and margin status (χ2 = 12. 36,P =0. 002). Multivariate analysis revealed that margin status was an independent risk factor (RR = 2. 219; χ2 = 9. 47,P = 0. 002) and radiotherapy was an independent protective factor ( RR = 0. 360;χ2 = 4. 95, P = 0. 026 ) for disease recurrence. When radiotherapy was delivered, the 10-year local recurrence rate decreased from 70. 1% to 20. 7% in patients with positive margin ( χ2 = 4. 22, P = 0. 040 )and decreased from 19.8% to 10.4% (χ2= 0.90, P= 0.344) in patients with negative margin.Conclusions Radical resection is the mainstay of treatment for aggressive fibromatosis. Postoperative radiotherapy can reduce the recurrent rate for patients with positive margin. In patients with negative margin,the role of radiotherapy should to be further evaluated in large clinical trials.     

腹壁侵袭性纤维瘤病21例临床分析

目的:探讨腹壁侵袭性纤维瘤病的临床特点和治疗方法。方法:回顾研究中国医科大学附属盛京医院2000-2010年间外科收治的21例腹壁侵袭性纤维瘤病例,包括初发18例,复发3例。术前3例复发患者确诊,9例疑诊。16例患者行广泛切除,5例患者行单纯肿瘤切除或切缘不足2cm。13例因肿瘤切除后腹部缺损较大用人工补片行腹壁重建。8例患者术后行放射治疗。结果:术后获随访18例,复发4例,复发率为22.2%,其中切缘大于2cm的14例患者中复发1例,切缘不足2cm且未行放疗的3例全部复发。7例加用放疗者均未复发。补片修补患者无复发及切口疝发生。结论:侵袭性纤维瘤呈侵袭性生长,复发率高,应提高对本病的认识。切缘阴性的手术是首选的治疗方法,放疗能降低术后复发率。     

胸腔内巨大侵袭性纤维瘤病二例报告

病例1：患者女,16岁。发现右腋下胸部肿块伴隐痛2年于2010—06—24入乐清市第二人民医院,无发热、咳嗽及活动后气促。体检：浅表淋巴结未及肿大,右腋下胸壁可触及境界不清肿块,质硬,不活动,无压痛,颈静脉无怒张,右肺叩诊呈实音,呼吸音消失,心界向左移位,律齐,未闻及杂音。     

头颈部侵袭性纤维瘤病放射治疗研究进展

侵袭性纤维瘤病（AF）为良性肿瘤,局部侵袭性强,治疗失败以局部复发为主。手术为头颈部AF的主要治疗手段,配合放疗能明显降低局部复发率。放疗适用于手术切缘阳性,手术后肉眼残留,手术近切缘,局部复发者,手术伤害大或不可手术切除者可行单纯放疗。放疗范围包括全部肿瘤或术床,外放范围依据周围解剖结构实际情况适当调整。放疗剂量为50~56 Gy。     

侵袭性纤维瘤20例临床分析

[目的]探讨侵袭性纤维瘤的临床特点及外科治疗原则.[方法]回顾分析1987年1月～2003年12月间收治的20例侵袭性纤维瘤患者的临床病理资料.行局部广泛切除术者13例,姑息性手术6例,1例放弃治疗,术后行辅助放疗2例,1例放弃治疗.[结果]全组病例5年复发率30.0%,手术切缘阳性者5年复发率83.3%,手术切缘阴性者5年复发率7.7%.全组无术后死亡病例.[结论]侵袭性纤维瘤具有侵袭性生长及复发率高等特点;局部广泛切除肿瘤,保证切缘阴性为该肿瘤外科手术原则.     

盆腹腔巨大复发侵袭性纤维瘤病放疗1例

侵袭性纤维瘤病(aggressive fibromatosis,AF),又称硬纤维瘤(desmoid tumor)或韧带样纤维瘤(desmoid-type fibromatosis),是一种罕见的软组织肿瘤.它属于一种良性疾病,但具有局部浸润性生长的特性,导致其手术完全切除困难,出现反复复发的征象.现将1例巨大复发侵袭性纤维瘤病患者的治疗情况报道如下.     

侵袭性纤维瘤病的非手术治疗

侵袭性纤维瘤病（aggressive fibromatosis,AF）是介于良恶性之间的交界性肿瘤,以局部浸润和高复发率为特点。AF的治疗既往以手术切除为主,但单纯手术复发率较高,盲目的手术往往造成患者机能或外形的损伤。近年来,包括内分泌治疗、手术、放疗、化疗、非甾体抗炎药物治疗、靶向药物、干扰素等多种治疗手段得到尝试,并取得一定疗效。此外,鉴于AF的惰性特征,观察等待也是多个行业指南推荐的一线方案。在AF的治疗决策上,必须综合考虑患者需求,注重机体功能和外形的保全,将非手术手段放到与手术同等地位加以考虑。     

侵袭性纤维瘤病诊治进展

侵袭性纤维瘤病不同于普通良性软组织肿瘤，表现为局部浸润生长．但未见转移。侵袭性纤维瘤病手术切除后，具有高局部复发率。放疗提高局部控制率，化疗应用于不宜手术和放疗患者是有益的。全文就侵袭性纤维瘤病的诊治作一综述。     

侵袭性纤维瘤病恶变为纤维肉瘤1例

1病案摘要患者男,69岁,因发现左上臂无痛性肿块6个月于2004年12月15日就诊。查体:局部皮肤无红肿、破溃,左上臂扪及5.0cm×3.0cm肿块,质硬,表面欠光滑,无触痛,活动度稍差,左上肢感觉、运动功能均正常。CT检查示左上臂软组织肿块影,密度均匀,未累及肱骨。予行左上臂肿物切除,术中见肿块大小约5.5cm×5.0cm×3.0cm,无完整包膜,边界不清,呈浸润性生长,术后病理示“侵袭性纤维瘤病”(图1),术后行一疗程46天30次总DT6000cGy放射治疗;2005年12月底出现左上臂肿胀感,查体:左上臂扪及11.0cm×7.0cm肿块,CT检查显示左肱骨上端背侧骨皮质菲薄,骨小…     

小儿侵袭性纤维瘤病19例临床分析

[目的]总结小儿侵袭性纤维瘤病的诊治体会.[方法]对1990年1月至2004年3月治疗的19例小儿侵袭性纤维瘤病资料进行回顾性分析.[结果]本组均经手术切除肿瘤及病理确诊,我院首诊的10例中,3例复发;9例外院复发病例经我院手术后,4例复发.[结论]小儿侵袭性纤维瘤病呈局部浸润性生长,手术是主要的治疗措施.     

小儿侵袭性纤维瘤病的病因及临床分析

小儿侵袭性纤维瘤病是一种少见的纤维组织增生,具有局部浸润生长、但不转移及恶变、术后易于复发之特点。我院近12年共收治侵袭性纤维瘤病患儿20例,手术48例次现将结果报告如下。一、临床资料1一般资料:本组男11例,女9例。初次手术年龄:1～5岁11例,5～10岁6例,10岁以上3例。肿瘤位于臀部12例,股部2例,腹部1例,颈部3例,颈及肩部2例。出生后即发现肿瘤1例,急性阑尾炎术后伤口处发现肿瘤1例,臀部注射后出现肿瘤2例,下蹲困难3例,余13例无记载。本组20例中无伴发家族性息肉及本病家族史。2术前诊断:术前伴发肢体活动受限13例,颈部肿瘤伴胸腔积液1…     

不可切除侵袭性纤维瘤病药物治疗进展

侵袭性纤维瘤病是一种发病率较低的交界性肿瘤,扩大切除是最有效的治疗方式,但其无包膜浸润性生长特性导致手术不易达到切缘阴性,而且累及重要脏器时常难以切除,甚至危及生命.对于难以切除、不能切除和切除后复发的患者,仍寄希望于药物治疗.药物治疗是腹腔内和多发性侵袭性纤维瘤病的一线治疗方案.目前,应用于侵袭性纤维瘤病的药物主要包括非细胞毒性药物和细胞毒性药物.本文就不可切除侵袭性纤维瘤病药物治疗进展做一综述.     

四肢侵袭性纤维瘤病的治疗体会

目的探讨四肢侵袭性纤维瘤病的临床特点和外科手术联合放疗的临床效果。方法回顾性分析43例四肢侵袭性纤维瘤病患者的临床资料,观察疾病的临床特点并对比单纯手术（24例）与手术联合放疗（19例）的治疗效果。结果单纯手术组复发率为33.3%,手术联合放疗治疗组复发率为21.1%。2组生存率均为100.0%,四肢功能评定均为良好。结论四肢侵袭性纤维瘤病首选外科手术治疗,术后放疗可以达到良好的局部控制效果,应结合患者实际病情及术后生活质量制定个体化方案。     

侵袭性纤维瘤病多学科综合诊疗研究进展CSCD

侵袭性纤维瘤病(aggressive fibromatosis,AF)是一种临床罕见的、起源于软组织的交界性肿瘤,尽管不具备远处转移能力,然而其局部侵袭性强,手术切除后复发率高,使临床治疗存在一定难度。近些年,随着对AF研究的不断深入,其治疗措施与管理模式发生了较大的变化。鉴于AF不可预测的自然病史,目前观察等待是指南推荐的一线治疗方案。此外,目前以手术根治性切除为主联合局部与全身系统性治疗等多学科综合诊疗模式可以在保留患者组织器官功能的同时,最大程度地控制肿瘤,延缓疾病的进展甚至达到根治。本文就AF的临床特征与诊疗进展展开综述。     

腹内韧带样型纤维瘤病1例

患者,男,27 岁。 因发现右下腹肿块一周于 2005 年 2 月入院。 腹部 MRI 示:后腹膜肿块,神经鞘瘤可能性大。 临床诊断:后腹膜肿块,恶性可能。 术中见肿块位于回肠近回盲部肠壁上,直径约 10cm。 行肿瘤及部分升结肠切除术。