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腹壁散发性硬纤维瘤:手术结果

Sporadic desmoid tumors of the abdominal wall: the results of surgery.

作者信息

Mussi Chiara E, Colombo Piergiuseppe, Lo Russo Chiara, Kasangian Anaid, Cananzi Ferdinando, Marrari Andrea, Morenghi Emanuela, De Sanctis Rita, Quagliuolo Vittorio

机构信息

Department of Surgical Oncology, Humanitas Clinical and Research Center, Milan - Italy.

Department of Pathology, Humanitas Clinical and Research Center, Milan - Italy.

出版信息

Tumori. 2016 Dec 1;102(6):582-587. doi: 10.5301/tj.5000552. Epub 2016 Sep 3.

Abstract

PURPOSE

Before the wait-and-see policy became the standard approach in abdominal wall desmoid tumors, surgery was performed on a systematic basis. Surgery remains indicated for progressing tumors but its extent is debatable. The abdominal wall is a common site of origin of sporadic desmoids, usually associated with a favorable prognosis. We analyzed the results of surgery at this specific site.

METHODS

Data from 33 patients affected by sporadic desmoid tumors of the abdominal wall (31 primary, 2 recurrent) consecutively treated at our cancer center between January 2000 and September 2013 were retrospectively studied.

RESULTS

Twenty-nine patients underwent surgery upfront and 1 after progression during the initial wait-and-see period. Prosthetic reconstruction of the abdominal wall was required in 28 patients. The average hospital stay was 5 days. Three patients developed surgical complications. Local recurrence-free survival was 90% at 5 and 10 years. Three patients had an uneventful childbirth during the follow-up after surgery.

CONCLUSIONS

Desmoid tumors of the abdominal wall have a favorable prognosis after surgical resection, which remains a safe and effective treatment. Wild-type tumors are common, whereas the incidence of S45F mutation in the beta-catenin gene is lower than in other anatomic sites. Upfront surgery may be considered in selected women who wish to bear a child.

摘要

目的

在等待观察策略成为腹壁硬纤维瘤的标准治疗方法之前,手术是按系统方式进行的。对于进展性肿瘤,手术仍然是必要的,但手术范围存在争议。腹壁是散发性硬纤维瘤的常见起源部位,通常预后良好。我们分析了在这个特定部位进行手术的结果。

方法

回顾性研究了2000年1月至2013年9月期间在我们癌症中心连续治疗的33例腹壁散发性硬纤维瘤患者(31例原发性,2例复发性)的数据。

结果

29例患者一开始就接受了手术,1例在初始等待观察期病情进展后接受了手术。28例患者需要进行腹壁假体重建。平均住院时间为5天。3例患者出现手术并发症。5年和10年的局部无复发生存率为90%。3例患者在手术后的随访期间顺利分娩。

结论

腹壁硬纤维瘤手术切除后预后良好,手术仍然是一种安全有效的治疗方法。野生型肿瘤很常见,而β-连环蛋白基因中S45F突变的发生率低于其他解剖部位。对于希望生育的特定女性,可以考虑一开始就进行手术。

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