未经评估:由于其罕见,目前缺乏针对侵袭性纤维瘤病(AF)的标准治疗指南。
UNASSIGNED:本研究旨在探讨根治性手术切除后腹部AF复发和生存的危险因素。
UNASSIGNED:2012年8月至2020年12月,对复旦大学附属上海公共卫生临床中心69例房颤的临床资料进行回顾性分析,肿瘤位于腹壁或腹腔。主要观察终点为无进展生存时间(PFS)和总生存时间(OS)。
未经授权:所有69例患者均获得显微镜下R0切除,10例(14.5%)局部复发,3人(4.3%)死亡。1、3、5和10年后的PFS率为96.8%,87.7%,78.8%,78.8%,分别。1年、3年、5年和10年后的OS率为100%,100%,92.9%,和81.3%,分别。在10例复发患者中,中位复发时间为17.6个月.合并家族性腺瘤性息肉病(FAP)和既往复发史是术后复发的独立危险因素。
未经证实:腹部房颤根治术后,局部复发率为15%。合并FAP和既往复发史是术后复发的独立危险因素。尤其是在FAP患者中,应进行R0和联合器官切除术,以最大程度地减少复发并改善预后。
未经证实:本研究确定了房颤复发的危险因素,并建议R0切除,尤其是合并FAP患者。不应普遍实施观望策略,根治性手术将为患有此类罕见疾病的患者带来临床益处。
UNASSIGNED: There are lacking standard treatment guidelines for aggressive fibromatosis (AF) because of its rarity.
UNASSIGNED: This study aimed to investigate the risk factors for recurrence and survival of abdominal AF after radical surgical resection.
UNASSIGNED: From August 2012 to December 2020, a retrospective analysis was conducted on the clinical data of 69 AF in Shanghai Public Health Clinical Center Affiliated to Fudan University, with the tumor locating either in the abdominal wall or in the abdominal cavity. The main observation end point was progression-free survival time (PFS) and overall survival time (OS).
UNASSIGNED: All 69 patients achieved microscopic R0 resection, 10 (14.5%) had local recurrence, and 3 (4.3%) died. The PFS rate after 1, 3, 5, and 10 years was 96.8%, 87.7%, 78.8%, and 78.8%, respectively. The OS rate after 1, 3, 5, and 10 years was 100%, 100%, 92.9%, and 81.3%, respectively. In 10 patients with recurrence, the median recurrence time was 17.6 months. Concomitant familial adenomatous polyposis (FAP) and history of previous recurrence were independent risk factors of post-operative recurrence.
UNASSIGNED: After radical surgery of abdominal AF, the local recurrence rate was 15%. Concomitant FAP and a previous history of recurrence were independent risk factors of post-operative recurrence. R0 and a combined organ resection should be performed especially in FAP patients to minimize the recurrence and improve the prognosis.
UNASSIGNED: The present study identifies the risk factors of recurrence in AF and suggests R0 resection especially in concomitant FAP patients. A wait-and-see strategy should not be generally implemented and radical surgery will bring clinical benefits to patients with such kind of rare disease.