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.     

151例韧带样瘤的疗效及预后分析

背景与目的:韧带样瘤的预后影响因素仍存在争论.既往大样本预后分析往往混杂有腹腔内韧带样瘤和手术不完整切除的情况.本文着重分析外科手术完整切除(R0,R1)的腹壁及腹壁外韧带样瘤的预后影响因素.方法:收集1987年1月-2007年12月经本院外科收治的198例韧带样瘤患者的资料,回顾性分析其中手术完整切除无肉眼残留的151例患者临床资料、随访其生存及复发情况.结果:中位随访时间为102个月,随访期间无患者死亡,总体生存率为100%.31例患者局部复发,复发率为20%.其中5年无复发生存率(recurrence free survival,RFS)为79%,10年RFS为78%.单因素分析表明,入院情况、性别、肿瘤部位及大小、病变数目、切缘情况是影响RFS的主要因素(P＜0.05).多因素分析表明,肿瘤大小与切缘情况是影响RFS的独立预后因素.辅助放疗并不能显著提高患者的局部控制率,但切缘阳性患者可能从中受益.结论:无论是原发还是复发腹壁及腹壁外韧带样瘤患者,都应在保全肢体功能的情况下,争取做到R0切除,腹壁外韧带瘤患者较易复发,若切缘阳性,治疗应该更加积极.病变数目是局部复发的独立预后因素,多发性韧带样瘤更易复发,诊治较棘手,临床应注意多中心发病的可能性.     

韧带样瘤的诊治进展及热点问题

韧带样瘤是一种深部筋膜或肌腱膜结构来源的低度恶性的纤维性肿瘤,发病率较低.腹壁外和腹壁韧带样瘤的治疗以外科手术为主.首选切缘2 cm以上的广泛根治性切除,确保切缘阴性;无法达广泛切除或切缘阴性时,首选保全功能性手术.切缘阳性、复发性患者或无法手术者可考虑辅助放疗.腹腔内韧带样瘤多见于家族结肠腺瘤息肉病患者,手术切除复发率高,目前推荐首选大剂量的三苯氧胺和舒林酸治疗.由于韧带样瘤自然病程多变,药物治疗等疗效多不确切,包含观察等待的个体化治疗方案有望成为韧带样瘤的一种新的治疗模式.     

腹壁韧带样瘤21例诊治分析

[目的]探讨腹锈切带样瘤的诊断和治疗。[方法]回顾性分析1995～2005年收治的21例经病理确诊韧带样瘤患者，术前均行彩超检查，治疗采用手术广泛切除肿瘤。[结果]21例患者以年轻经产妇及有手术史者多见，因发现肿块就诊，彩超显示无包膜、实质性肿块，病理检查有特征性表现，行广泛切除术后复发5例，复发率23.81％。[结论]年轻经产妇及有于术史患者易患腹壁韧带样瘤，彩超有助于术前定位及明确浸润范围，确诊需病理检查，治疗首选肿瘤广泛切除。     

腹壁韧带样纤维瘤53例病例分析

 目的　探讨腹壁韧带样纤维瘤的临床特点和治疗 ;方法　回顾分析 1964～ 1997年间外科收治的 53例腹壁韧带样纤维瘤 ;结果　 53例腹壁韧带样纤维瘤行广泛切除术 32例 ,根治切除术 19例 ,姑息切除术 2例 ,术后合并放射治疗 1例 ,全组病例复发率 2 0 8%,本院治疗者复发率 3 8%( 2 53) ;结论　腹壁韧带样纤维瘤呈侵袭性生长的特性 ,治疗应按低度恶性软组织肿瘤处理。治疗首选外科手术 ,配合放疗、化疗和内分泌治疗可提高疗效。     

韧带样型纤维瘤病手术联合放疗疗效观察

目的:观察韧带样纤维瘤病手术联合放疗的疗效.方法:总结我院收治的12例手术联合放疗韧带样纤维瘤病的3年无病生存率.结果:其中位于四肢8例,胸壁2例,腹壁2例.均行手术和术后放疗,手术病理证实为韧带样纤维瘤病,其中波形蛋白阳性10例,阴性2例.术后随访3年,1例复发,其他11例均无病生存,无病生存率83.33%.结论:手术联合放疗可提高韧带样纤维瘤病的无病生存率.     

韧带样型纤维瘤病手术联合放疗疗效观察

目的：观察韧带样纤维瘤病手术联合放疗的疗效。方法：总结我院收治的12例手术联合放疗韧带样纤维瘤病的3年无病生存率。结果：其中位于四肢8例,胸壁2例,腹壁2例。均行手术和术后放疗,手术病理证实为韧带样纤维瘤病,其中波形蛋白阳性10例,阴性2例。术后随访3年,1例复发,其他11例均无病生存,无病生存率83.33%。结论：手术联合放疗可提高韧带样纤维瘤病的无病生存率。     

25例妇科复发性恶性肿瘤三维适形放疗观察

[目的]观察复发性妇科恶性肿瘤三维适形放疗的疗效和副作用。[方法]25例复发性妇科恶性肿瘤接受三维适形放疗,其中外院手术放疗后复发转移16例,本院初次治疗9例。单纯适形放疗13例,先盆腔大野放疗再加适形放疗12例。[结果]全组有效率76%(19/25),CR9例,PR10例,13例单纯适形放疗中CR7例,PR3例,12例常规加适形放疗中CR2例,PR7例。目前存活16例,无瘤存活9例,带瘤存活7例。全组总中位存活28.5个月,复发后中位存活11.4个月。血液学毒性轻到中度,无肝肾功能损害及膀胱炎,少数患者有放射性直肠炎。[结论]三维适形放疗治疗复发性妇科恶性肿瘤具有明显剂量分布优势,肿瘤缓解率高,副反应低。单纯适形放疗显示疗效优势。     

巴氏腺腺样囊性癌临床病理分析

目的 探讨巴氏腺腺样囊性癌的临床病理特征和治疗方法.方法 回顾性分析6例巴氏腺腺样囊性癌患者的临床及病理资料.6例患者的中位年龄为40.5岁(30～54岁).主要治疗方式为手术切除,其中单纯外阴肿物剥除术1例,外阴根治性切除术加双侧腹股沟淋巴结清扫或活检术4例,外阴局部扩大切除术加双侧腹股沟淋巴结活检术1例.有2例患者术后补充放疗.6例患者均随访至2009年4月1日,中位随访时间124.5个月(8～241个月).结果 6例巴氏腺腺样囊性癌均经病理确诊,肿瘤细胞呈筛状排列及侵犯神经是其典型的病理特点.术后病理显示,切缘阳性2例,阴性1例,邻近肿瘤1例,不详2例.腹股沟淋巴结阴性5例,不详1例.有4例患者复发,其中3例局部复发,后出现肺转移;1例仅出现肺转移.在复发患者中,死亡1例,生存时间为135个月;另3例患者分别带瘤生存120、30和36个月,总生存时间分别为241、128和103个月.2例无复发患者无瘤生存8个月和121个月.结论 巴氏腺腺样囊性癌生长缓慢,患者长期预后较好,但容易局部复发和肺转移.首选治疗方法为手术切除,对于术后切缘阳性、局部浸润较深或侵犯神经者以及复发无法手术者可行辅助放疗或姑息性放疗.     

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。     

硬纤维瘤的临床特点--附100例病例报告

目的：探讨硬纤维瘤的临床特点和治疗方法。方法：回顾分析我院１９６４－１９９７年间外科收的１００例硬纤维瘤,包括腹壁硬纤维瘤５３例,腹壁外硬纤维瘤４７例。结果：全组１００例硬纤维瘤行广泛切除术６８例,根治切除术２７例,术后合并放疗１２例,全组病例复发率３６％,本院治疗复发率１０．８％,１例病人死于肿瘤肺转移。结论：硬纤维瘤呈侵袭性生长,复发率高,治疗应按低度恶性软组织肿瘤处理。外科手术是主要的治疗方     

Abdominal wall desmoid tumor--analysis of 42 patients

Forty two patients with abdominal wall desmoid tumor, including one Gardner's syndrome, are reported. All patients were female except one. The tumor occurred in various sites in the abdominal wall, 66% in the lower abdominal wall. The fascia, sheath and muscle layer were chiefly involved and a very large tumor could invade peritoneum and viscera. This tumor shows aggressive growth and is prone to recurrence. Surgery is the treatment of choice. Local recurrence rate was 5.5%. Abdominal wall desmoid tumor associated colonic polyposis is named Gardner's syndrome. The authors emphasize that a local extended resection should be performed with a safety margin at least 2-3 cm beyond the tumor. Peritoneum, if involved, should be resected together with the primary focus.     

腹壁韧带样瘤的临床与超声分析

为了探讨二维超声及彩色多普勒血流显像(CDFI)对腹壁韧带样瘤的诊断价值,对27例经病理确诊的腹壁韧带样瘤的二维声像图及彩色多普勒血流显像进行分析。27例患者中26例单发,1例多发有2个肿块。肿块最大径2.5~13cm,平均7.3cm,肿块长轴与累及的肌肉、肌腱方向一致;肿块边缘规则,与肌肉分界清晰12例,模糊不清15例。CDFI检查19例肿块内仅见稀疏点状或短棒状血流信号,8例可见条状或较丰富血流信号。初步研究结果提示,二维超声及彩色多普勒血流显像对腹壁韧带样瘤的诊断及鉴别诊断具有一定的临床价值。     

The therapeutic procedure for desmoids in patients with diffuse polyposis of the large intestine]

Desmoid was diagnosed in 54 out of 632 patients who had been operated on for diffuse polyposis. The age of patients with desmoid ranged from 18-61 years. Twenty of them had desmoid in the anterior abdominal wall whereas 30--in the small intestine mesentery or retroperitoneal space. Dissection was carried out in 14 out of 18 patients with anterior abdominal wall desmoid. Recurrence was registered in 6 patients. Dissection was attempted in 12 out of 26 patients with intraabdominal desmoid but radical procedure could be performed in 5 only. In a group of 4 patients with synchronous desmoid of both sites, both tumors were removed in one patient only whereas in the other 3--only anterior abdominal wall tumor could be dissected.     

腹壁肠造口周围皮肤糜烂的防治

（目的）探讨腹壁肠造口周围皮肤糜烂的预防和治疗。（方法）对1990年至1997年158例腹壁造口病人中，发生造口周围皮肤糜烂的6例病人的治疗进行总结。（结果）采用中西医结合治疗，6例患者均获治愈。（结论）造口周围皮肤湿疹，多因粪便长期污染皮肤所致，控制腹壁肠造口规律性排便，可预防湿疹发生。采用中西医结合局部用药，湿疹均可治愈。     

手助腹腔镜诊治不明来源腹部巨大肿瘤临床体会

[目的]探讨手助腹腔镜诊治不明来源腹部巨大肿瘤的可行性和优越性。[方法]回顾性分析2例手助腹腔镜诊治不明来源腹部巨大肿瘤病例的临床资料。[结果]2例成功手术。术后病理诊断分别为十二指肠间质瘤和肾血管平滑肌脂肪瘤，肿瘤大小分别为25．Ocm×20．0cm×l5．Ocm及15．5cm×12cm×10cm，无严重并发症，均治愈出院。[结论]术前准备充分，遵守肿瘤手术原则，手助腹腔镜诊治部分不明来源的腹部巨大肿瘤应是安全可行的，并保留了微创外科的优点。     

A nation‐wide study comparing sporadic and familial adenomatous polyposis‐related desmoid‐type fibromatoses

Desmoid‐type fibromatoses are neoplasms of fibroblastic origin, occurring sporadically or associated with familial adenomatous polyposis (FAP) coli. By comparing sporadic and FAP‐associated desmoid‐type fibromatoses, we tried to identify clinical characteristics, which may indicate FAP. Histopathology data of all Dutch patients with desmoid‐type fibromatoses diagnosed between 1999 and 2009 were retrieved from PALGA, the nation‐wide network and registry of histopathology in the Netherlands. For calculation of incidence rates, person‐years from the general matched population were used. Based on polyp counts in pathological records, the cohort was divided into a FAP group and a non‐FAP group. Patient‐ and tumor characteristics were compared between the two groups. A total number of 519 patients older than 10 years with a confirmed diagnosis of desmoid‐type fibromatoses were included. Thirty‐nine (7.5%) desmoid patients were documented of having FAP. The incidences of sporadic and FAP‐related desmoid‐type fibromatoses were 3.42 and 2,784 per million person‐years, respectively. The majority of FAP patients developed desmoid‐type fibromatoses after the diagnosis of FAP. Having FAP was associated with male gender [odds ratio (OR) 2.0, p = 0.034], desmoid diagnosis at an earlier age (mean 36 vs. 42 years, p = 0.031), and desmoid localization intra‐abdominally (OR 18.9, p ≤ 0.001) or in the abdominal wall (OR 4.8, p ≤ 0.001), compared to extra‐abdominal desmoid localization. In conclusion, patients with desmoid‐type fibromatoses are at risk of underlying FAP. Especially cases with desmoid localization intra‐abdominal or in the abdominal wall, and all patients younger than 60 years, have a substantial increased risk and should be referred for colonoscopy.     

Inflammatory pseudotumor of the liver

Inflammatory pseudotumor of the liver is not common. Of the 76 patients who underwent hepatectomy in our hospital from 1986-1988, 4 had inflammatory pseudotumor of the liver. It comprised 5.2% of patients subjected to liver resection during the same period. These 4 patients were misdiagnosed as primary liver cancer and desmoid tumor of the upper abdominal wall by ultrasonography and CT scan before operation. The inflammatory pseudotumor of the liver had long clinical course, less symptoms, and clear capsule, tenacity in nature and no liver cirrhosis on operation. The patients received modified hepatectomy without any postoperative complications. Hepatic resection is the treatment of choice. If contra-indicated, steroid can be used.