手术切除联合一期腹膜前间隙补片修补治疗腹壁韧带样纤维瘤的疗效

目的探讨手术切除联合腹膜前间隙补片修补治疗腹壁韧带样纤维瘤的临床疗效。方法采用回顾性横断面研究方法,收集2005年4月至2018年7月四川大学华西医院收治的19例腹壁韧带样纤维瘤患者的临床资料,患者均行腹壁韧带样纤维瘤手术切除联合补片修补术。观察指标:(1)手术及术后情况:合并症、肿瘤大小、病理分型、住院时间、术后并发症情况;(2)随访情况:腹壁韧带样纤维瘤远期并发症情况及术后放化疗情况。采用电话、门诊或住院方式对患者进行为期12个月的随访,了解患者肿瘤复发及远期并发症发生的情况。随访时间截至2019年7月。结果所有患者均行根治性肿块切除术和腹壁修补术,未进行术后放化疗。4例免疫组化结果显示肿瘤细胞均呈β-catenin表达,SMA(+)3例,Desmin(灶性+)1例。无术后并发症,随访期间内无肿瘤复发,也无远期并发症发生;住院时间9～22 d,平均14.8 d。结论韧带样纤维瘤是一种交界性肿瘤,易复发,首选外科手术治疗,术中扩大切除,借助冰冻切片,保证切缘阴性。对于切除后造成的腹壁缺损,应对腹壁进行一期重建。     

ADDIN CNKISM.UserStyle手术切除联合一期腹膜前间隙补片修补治疗腹壁韧带样纤维瘤的疗效

目的探讨手术切除联合腹膜前间隙补片修补治疗腹壁韧带样纤维瘤的临床疗效。 方法采用回顾性横断面研究方法,收集2005年4月至2018年7月四川大学华西医院收治的19例腹壁韧带样纤维瘤患者的临床资料,患者均行腹壁韧带样纤维瘤手术切除联合补片修补术。观察指标：（1）手术及术后情况：合并症、肿瘤大小、病理分型、住院时间、术后并发症情况;（2）随访情况：腹壁韧带样纤维瘤远期并发症情况及术后放化疗情况。采用电话、门诊或住院方式对患者进行为期12个月的随访,了解患者肿瘤复发及远期并发症发生的情况。随访时间截至2019年7月。 结果所有患者均行根治性肿块切除术和腹壁修补术,未进行术后放化疗。4例免疫组化结果显示肿瘤细胞均呈β-catenin表达,SMA（＋）3例,Desmin（灶性＋）1例。无术后并发症,随访期间内无肿瘤复发,也无远期并发症发生;住院时间9～22 d,平均14.8 d。 结论韧带样纤维瘤是一种交界性肿瘤,易复发,首选外科手术治疗,术中扩大切除,借助冰冻切片,保证切缘阴性。对于切除后造成的腹壁缺损,应对腹壁进行一期重建。     

腹壁韧带样瘤六例诊治分析及文献复习

目的探讨腹壁韧带样瘤的诊断和治疗。方法回顾性分析攀钢集团总医院1999年3月至2015年1月收治的6例腹壁韧带样瘤病人的临床资料。病人以腹部包块入院,无特异性的临床表现。治疗上均选择以手术治疗为主,术中保留皮肤及皮下组织,沿肿瘤边缘完整游离肿瘤,距离肿瘤边缘大于2 cm完整切除肿瘤,将切除的肿瘤送冰冻病理检查,若切除的肿瘤边缘为阳性,则继续扩大切除范围。手术后若腹壁缺损较大,则用补片予以修补,术后予以放疗。结果 6例病人中女性5例,均为经产妇,1例为男性;均因为发现腹壁包块入院就诊,术前经过彩超或CT检查证实肿块位于腹壁,肿瘤最长径在2.7~7.9 cm之间,平均为5.6 cm。6例病人均经手术广泛切除治疗,其中2例接受术后放疗。术后随访7~361个月,复发者1例(未接受术后放疗),复发率为16.67%。结论腹壁韧带样瘤为临床少发疾病,发展缓慢,无特异性临床表现,以女性好发,治疗上以外科手术为主,术后放疗可降低复发率。     

人工补片应用于韧带样瘤切除后腹壁无张力修补

目的对比腹壁韧带样瘤切除术后直接缝合及无张力修补方法的临床效果。方法 1990年—2002年收治7例腹壁韧带样瘤,行肿块切除腹壁直接缝合;2003年—2010年收治9例腹壁韧带样瘤患者,行肿瘤广泛切除术,并使用人工补片进行腹壁缺损修补。结果直接缝合患者术后平均72 h后下床活动,平均住院14 d;无张力修补患者术后6~12 h均可下床活动,平均住院9 d。前者随访7年~20年,术后出现一例局部复发,一例切口疝,均行二次手术;后者随访6个月~7年无肿瘤复发及切口疝发生。结论腹壁韧带样瘤治疗需行肿块扩大切除术,术中使用人工材料进行腹壁缺损修补可使患者早期下床活动,减少住院时间,降低术后并发症率及控制复发。     

腹腔镜下腹壁侵袭性纤维瘤根治性切除加一期腹壁重建修复1例报告

<正>腹壁侵袭性纤维瘤病好发于腹壁肌层与筋膜鞘,尤以下腹部多见,又称腹壁韧带样纤维瘤、腹壁硬纤维瘤等^[1]。本病发病率为 2 /100 万 ～ 4 /100 万^[2],发病原因尚不清楚,可能与遗传、内分泌异常、物理创伤等有关^[3]。男、女发病比例为 1: 7,尤以育龄期经产妇多见^[4]。其生物特征介于良性成纤维细胞瘤与纤维肉瘤之间,1950 年 Wills 将其界定为交界性肿瘤,得到广泛认同。其治疗包括手术、放疗、化疗、内分泌治疗及保守性治疗等,以开放扩大范围切除的手术治疗为主^[5-6]。目前尚缺少利用腹腔镜切除腹壁侵袭性纤维瘤的报道。笔者将大连医科大学附属第一医院普通外科团队完成的 1 例腹腔镜下腹壁侵袭性纤维瘤根治性切除加一期腹壁重建修复手术进行总结讨论,以便于临床及研究人员对该病有新的认识。患者女,24 岁,因“发现右下腹壁包块 7 天”于 2018 年 10 月 23 日入院。患者 7 d 前无意中发现右下腹壁包块,约鹅蛋大小,包块固定,无疼痛、发热等不适,...     

活骨移植治疗四肢骨肿瘤切除后长段骨与关节缺损

目的:探讨四肢骨肿瘤的广泛或边缘切除后,长段骨关节缺损的修复方法及其疗效。方法:四肢骨肿瘤切除术后骨缺损14例,男10例,女4例;年龄4~37岁。骨巨细胞瘤4例,骨化性纤维瘤1例,非骨化性纤维瘤2例,韧带样纤维瘤1例,骨纤维结构不良3例,侵袭性骨母细胞瘤1例,软骨母细胞瘤1例,瘤样病损致骨溶解1例。14例四肢侵袭性良性骨肿瘤行肿瘤广泛或边缘切除,应用吻合血管的腓骨、带腓骨头腓骨近端和带旋髂深血管的髂骨瓣移植修复骨缺损、重建桡腕关节和重建肩关节。移植体行简单内固定加外固定。术后定期复查X线片、多普勒血管超声,其中3例行ECT核素骨扫描,并作关节功能评定。结果:随访3个月~5年,13例均于手术后3个月后达Ⅰ期愈合,其中1例因内固定松动再次手术而愈合。1例肿瘤局部复发,再次行肿瘤切刮与植骨术后愈合;2例畸形愈合行截骨矫形后愈合。关节功能评定:优9例,良3例,差2例。结论:骨肿瘤切除后行骨移植修复骨缺损、重建关节,手术切除彻底,复发率低,植骨愈合可靠,重建后的关节功能良好。     

60例韧带样型纤维瘤病临床病理特点及预后分析

目的探讨韧带样型纤维瘤病临床、影像学及病理特点,为临床诊治及预后提供依据。方法收集2002年1月至2013年12月经本院收治的60例韧带样型纤维瘤患者的临床资料,其中男26例,女34例,男女比例1︰1.3。患者年龄8个月~55岁,中位年龄25岁。发病部位位于臀部25例,肩部5例,背部4例,上肢4例,手4例,足部4例,颈部3例,腹部2例,锁骨部位2例,胸壁2例,肘部2例,髂窝1例,小腿2例。60例患者均接受了手术治疗,33例复发患者均行扩大或广泛切除术,其中1例行右上肢截肢术,其余27例初治患者20例行扩大切除术,7例行边缘切除术。结果 27例原发初治患者,2例术后复发。33例复发再治患者,8例复发,以上均未见转移。结论韧带样型纤维瘤病是一种临床表现以肢体局部疼痛、质硬肿块为特点,病理以浸润侵袭周围正常组织为特征的罕见良性肿瘤。肿瘤切缘阳性或边缘性切除是导致肿瘤复发的重要原因,无论是原发还是复发韧带样纤维瘤患者,都应在保全肢体功能的情况下,争取做到扩大切除或广泛切除时,在无法保证肿瘤完整切除时,应进行术后放疗的进一步治疗并密切随访。     

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韧带样瘤来源于深部结缔组织主要是肌肉内结缔组织及其被覆的筋膜或腱膜的单克隆纤维母细胞性肿瘤。其中腹壁外来源的就称为腹壁外韧带样瘤。1923年由Nichols首次报道命名。本病在形态上表现良性，但其生物学行为属于低度恶性，呈侵袭性生长、易复发，但无转移。故又称侵袭性纤维瘤病、非转移性纤维肉瘤、腹壁外纤维瘤病。     

体表软组织巨大神经纤维瘤的手术治疗

目的 探索手术治疗体表软组织巨大神经纤维瘤的方法和疗效。方法 自2004年1月至2013年12月,共治疗21例体表软组织巨大神经纤维瘤患者。根据瘤体的部位、侵犯程度和局部解剖等情况,采用直接手术切除部分瘤体皮瓣修复、完整切除瘤体植皮修复、扩张器治疗逐步切除瘤体、介入栓塞主要营养动脉后切除瘤体等方法治疗。结果直接手术切除部分瘤体10例,均行皮瓣修复创面;直接手术完整切除瘤体4例,均行植皮修复;扩张器治疗切除头部瘤体1例;介入栓塞主要营养动脉后切除瘤体6例。17例手术切口一期愈合,4例患者手术切口愈合不良,经换药后愈合。所有患者术后局部外形明显改善,肢体功能得以保留并有所提高。结论 手术治疗巨大神经纤维瘤能有效减轻瘤体负荷,改善外形和功能,提高患者生活质量。对于合适的患者应用扩张器治疗和术前进行营养动脉栓塞能提高手术效果,保障手术安全。     

腹壁韧带样瘤16例临床分析

目的 探讨腹壁韧带样瘤的临床特点和治疗方法.方法 回顾性分析1998年1月至2010年10月收治的16例腹壁韧带样瘤患者的临床资料,全组患者均经手术治疗.结果 本组16例患者均行广泛切除术,复发2例,复发率12.5%(2/16).结论 腹壁韧带样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.     

Abdominal wall reconstruction with intraperitoneal prosthesis in desmoid tumors surgery

Desmoid tumor (DT), also known as aggressive fibromatosis, is a rare soft tissue neoplasm. For those tumors localized in the anterior abdominal wall, radical resection and reconstruction with mesh are indicated. Due to the rarity of this disease, there have been no randomized trials, but in reported retrospective series, although it is considered a benign lesion, it is clear that local recurrence is not uncommon. Records from seven consecutive patients (1 man, 6 women; mean age 35 years, range 25-60 years) presenting with desmoid tumors of the anterior abdominal wall were analyzed. In all cases the surgical strategy was the same: wide surgical excision and immediate plastic reconstruction with ePTFE mesh after intraoperative confirmation by frozen section of disease-free margins >1 cm. No immediate postoperative complications were recorded, and no patients developed recurrence after a median follow-up period of 60 months. The long-term mean of global health status recorded was 100%. Radical resection aided by intraoperative margin evaluation by frozen sections, followed by immediate mesh reconstruction, is a safe procedure and can provide a definitive cure without functional limitations for patients with desmoid tumors of the anterior abdominal wall.     

Desmoid Tumors of the Anterior Abdominal Wall: Results from a Monocentric Surgical Experience and Review of the Literature

Background  Desmoid tumor, also known as aggressive fibromatosis, is a rare soft tissue tumor. For those cases localized in the anterior abdominal wall, radical resection and reconstruction with a mesh is indicated. Because the rarity of the disease, randomized trials are lacking, but in reported retrospective series, it is clear that although it is considered a benign lesion, local recurrence is not uncommon. Methods  We analyzed the records of 14 consecutive patients (3 men, 11 women, mean age 36 years, range 25–51 years) with desmoid tumor of the anterior abdominal wall treated at the European Institute of Oncology. The surgical strategy was the same in all cases: wide surgical excision and immediate plastic reconstruction with mesh after intraoperative confirmation by frozen sections of disease-free margins of >1 cm. We considered long-term outcomes by using the European Organization for the Research and Treatment of Cancer QLQ-C30 as an instrument to evaluate the overall quality of the treatment delivered to these patients. Results  No immediate postoperative complication was registered, and no patient developed recurrence after a median follow-up period of 55 months. Two women experienced mesh bulging within 1 year after the operation. The long-term mean global health status registered was 97 out of 100. Conclusions  Radical resection aided by intraoperative margin evaluation via frozen sections followed by immediate mesh reconstruction is a safe procedure and can provide definitive cure without functional limitations for patients with desmoid tumors of the anterior abdominal wall.     

Desmoid tumors of the abdominal wall.

Four cases of desmiod tumors of the anterior abdominal wall recently treated at the University of Chicago Hospitals and Clinics are presented. From our experience we conclude: 1. Inadequate excision of desmoid tumors results in local recurrence. 2. Adequate excision of desmoid tumors ot the abdominal wall sometimes necessitates creation of an abdominal wall defect too large for primary closure. 3. Marlex mesh has been used successfully in reconstruction of the abdominal wall following adequate excision of desmoid tumors. 4. One patient (Case 4) with an abdominal wall desmoid tumor associated with familial polypisis and mesenteric fibromatosis has benefited greatly following wide excision of abdominal wall and reconstriction using Marlex mesh, despite the fact that the mesenteric fibrous tumor was unresectable.     

Abdominal wall and foot reconstruction after extensive desmoid tumor resection with free tissue transfer

BACKGROUND: Desmoid tumors are rare connective tissue tumors, also referred to as deep, aggressive fibromatosis. Although histologically benign, they show an invasive growth behavior and have a high local recurrence rate. METHODS: The treatment of choice is surgical resection with wide negative margins, while the use of radiotherapy remains controversial. Wide resection of greater desmoid tumors may result in considerable defects and functional impairment. Few papers discuss different options for defect coverage after desmoid tumor resection. Two cases of extensive desmoid tumors, one at the trunk, one at the foot, both with compromised wound margins due to multiple previous surgeries, are presented. To achieve a stable and functional soft tissue cover, the defects were treated with microvascular soft tissue transfer (one free latissimus dorsi, one free radial forearm flap). RESULTS: Both flaps healed uneventfully and patients regained full function of the abdominal wall and foot, respectively. CONCLUSIONS: The presented cases demonstrate the efficacy of free flap coverage as an ultimate therapeutic option in selected cases of critical defects after extra-abdominal desmoid tumor resection. Furthermore, free flaps provide a well vascularized ground for adjuvant radiotherapy.     

腹壁韧带样纤维瘤的术后腹壁重建的治疗研究

目的探讨人工补片在韧带样纤维瘤病术后腹壁重建中的临床疗效。 方法回顾性分析2012年2月至2016年2月,首都医科大学附属北京朝阳医院收治的11例腹壁韧带样纤维瘤病患者的临床资料,所有患者行根治性手术,术后腹壁缺损使用人工补片行腹壁重建,分析其临床疗效。 结果所有患者均使用人工补片行腹壁重建,平均手术时间（97±33）min,平均住院时间（19.2±3.1）d,所有患者术后恢复良好,伤口Ⅰ期愈合,无伤口感染及局部异物感,无死亡患者,出现皮下积液者1例,经穿刺抽吸后治愈。随访时间7～60个月,平均随访时间（31.8±18.5）个月,肿瘤复发1例,无死亡患者,无补片感染、慢性疼痛及局部异物感等补片相关并发症,无肠梗阻及切口疝发生。 结论人工补片用于腹壁重建具有良好的临床疗效。     

Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review

Aggressive fibromatosis in children is a rare, benign condition that is locally infiltrative and destructive. It often presents as a rapidly growing, painless lump in the head and neck region. To date, only small series and case reports have been reported, and the management of the condition remains unclear. Recently, nuclear β-catenin expression has been suggested as a tumor-specific marker for aggressive fibromatosis (desmoid).

Aim

The aims of the study were to review our experience of the presentation, management, and treatment outcome of pediatric aggressive fibromatosis in the head and neck and to identify the presence of the desmoid tumor marker β-catenin within this population.

Method

The study was conducted as a retrospective case review of children diagnosed with aggressive fibromatosis in the head and neck for a period of 20 years and a review of the literature. Pathologic review of the original tumor specimens was undertaken for evidence of positive tumor margins and presence of nuclear β-catenin expression.

Results

A total of 10 patients (6 males, 4 females) were identified. The age at presentation ranged from 12 months to 14 years. In total, 8 patients were treated with surgery alone. This included 7 patients with extension of the tumor to the resection margin; all had good long-term outcomes with no disease progression. Two patients received chemoradiotherapy, one as primary treatment, and the other as adjuvant treatment after gross incomplete resection. Both resulted in poor outcomes requiring further treatments.Within our series of pediatric fibromatosis, only 4 cases (40%) had positive results for any nuclear β-catenin expression, and 6 (60%) of 10 patients had negative results for β-catenin.

Conclusion

Our experience is that total gross resection and preservation of form and function is of higher priority than achieving a negative resection margin. Pediatric fibromatosis though aggressive is still a benign condition, and careful thought should be taken before considering adjuvant chemoradiotherapy. Nuclear β-catenin expression should not be considered a specific tumor marker for pediatric aggressive fibromatosis of the head and neck. Pediatric aggressive fibromatosis in this region may be a distinct subtype of desmoid tumor from its adult form.     

Monströse retrovesikale Raumforderung als Zufallsbefund

We report the case of a 57-year-old patient with a huge, retrovesical, aggressive desmoid fibromatosis detected incidentally during preoperative staging for radical prostatectomy. The tumor was locally invasive consisting of monoclonal and fibroblastic proliferations. The etiology of such tumors is not well known but they are mostly sporadic and aggressive fibromatosis is sometimes associated with familial adenomatous polyposis (FAP) or Gardner’s syndrome. Such desmoid tumors are slow growing and locally invasive but lack metastatic potential. Complete resection with wide margins is the therapy of choice and the tendency to recur is high. Radiotherapy following surgery is controversial. We discuss the pathogenesis, clinical manifestations and therapy of such rare tumors.     

Transplantation for the treatment of intra-abdominal fibromatosis

MATERIALS AND METHODS: During the last 9 years we treated 14 patients with a diagnosis of intra-abdominal fibromatosis. The 11 patients who received an intestinal allograft included isolated intestine (n = 6), liver-intestine (n = 1), intestine-kidney (n = 1), multivisceral (n = 1), multivisceral-kidney (n = 1), multivisceral-no liver (n = 1). Three patients received an intestinal autograft after partial abdominal evisceration and ex vivo tumor resection. Three patients additionally underwent an abdominal wall allograft. RESULTS: At follow-up until August 2004, all autotransplant patients are alive. Four intestinal transplant patients died within the first postoperative month. There were three graft losses. A patient who lost his graft early postoperatively was retransplanted but died of sepsis shortly there after. Two more patients lost their graft due to severe rejection and were retransplanted successfully. Two patients developed desmoid tumor recurrence in their abdominal or thoracic wall. Ten patients are alive 1 to 9 years posttransplantation. Nine have fully functioning grafts and one patient requires TPN supplementation at night due to dysmotility of her autograft. CONCLUSION: Intestinal allo-, or autotransplantation combined with transplantation of the abdominal wall can be lifesaving for patients suffering from extensive intra-abdominal fibromatosis.     

腹壁子宫内膜异位症相关性透明细胞癌一例及文献回顾

目的探讨剖宫产术后腹壁子宫内膜异位症（AWE）相关性透明细胞癌（CCC）的临床特点、治疗方法和预防措施。 方法搜集近10年AWE后恶性病变的相关资料结合1例相关患者的诊疗过程,对资料进行系统回顾分析。 结果共检索出410篇文献,最终25篇（其中英文20篇,中文5篇）文献符合纳入标准,纳入31例剖宫产后AWE发生CCC患者。其中12例行单纯肿瘤切除,17例同时行肿瘤切除和附加手术,2例未行手术治疗。接受化疗的患者为21例,放疗者6例,同时接受放化疗者5例。2例接受了术前新辅助化疗。12例患者出现肿瘤的扩散或转移。23例有随访信息,随访期间死于疾病者4例,带病存活者8例,无疾病迹象者11例。本文报道1例AWE后发生CCC的患者,行病灶及部分腹壁切除后使用补片进行腹壁重建治疗,术后恢复良好。 结论AWE后发生CCC恶变的情况近几年有增加的趋势。目前对此类恶变过程缺乏有效的预防与观察手段,对AWE者应早期行病变切除手术。对发生恶变者,应行包括病灶在内的腹壁整块切除手术,形成的缺损可行包括应用补片的腹壁重建手术治疗。