The infrequent large pelvi-perineal tumors as a surgical dilemma: en bloc resection and long-term results

IF 0.5 4区 医学 Q4 OBSTETRICS & GYNECOLOGY European journal of gynaecological oncology Pub Date : 2023-01-01 DOI:10.22514/ejgo.2023.052
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Abstract

To assess long-term results of surgical resections of the sporadic extra-large pelvi-perineal soft tissue tumors, a retrospective series of patients referred to the National Cancer Institute 2001–2022 (tertiary care cancer university hospital in Egypt) was designed. Thirteen patients (3 males) averaged age 57 years had large 29 cm (18–38) pelvi-perineal tumors (10 to the paraanal ischiorectal spaces and 3 to the vulva) plus upper intra-abdominal extensions in 9 patients (69%). Symptoms were nonspecific with delayed presentation that averaged 22-month. The entire underwent combined open abdomino-perineal approach to widely en masse resect tumors plus infiltrated organs. No downsizing hormonal treatment was offered preoperative. Main outcome measures were disease free survival, recurrence pattern and salvage. Extensive pelvic and perineal tumor resection is tough and meticulous but straightforward with minor (Clavien-Dindo Classification (CDC) grade I & II) early and delayed morbidity. Resection extends to hysterectomy, vaginectomy and vascular resection. Histopathology plus immunohistochemistry showed 9-aggressive angiomyxoid tumors (AA), 2-fibromatosis and 2-neurofibromas (NF). Margins of resection are all adequate (R0) except for 2 (Resection margin 1 (R1)). After 50-month median follow up, 8/13 total series (61.5%) were surviving free of disease. 4/9 of AA (44%) had local perineal and/or pelvic recurrences (13–37 months) and all were amenable to curative salvage resections; while, 1/2 patients with fibromatosis died of disseminated peritoneal relapses. No systemic metastases are noticed. Extensive tumors meticulous surgery with experienced pelvic dissection and resection could offer alone long term cure even after recurrences with minor morbidity in a good percent. Fertility sparing resection is not oncologically safe because of the frequent uterine and ovarian invasions.
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罕见的盆腔会阴大肿瘤作为手术困境:整体切除和长期效果
为了评估散发性特大型盆腔会阴软组织肿瘤手术切除的长期效果,设计了2001-2022年国家癌症研究所(埃及三级保健癌症大学医院)的回顾性患者系列。13例患者(3例男性)平均年龄57岁,盆腔会阴肿瘤大,29厘米(18-38厘米)(10例在肛门旁坐骨直肠间隙,3例在外阴),9例(69%)伴有上腹内扩张。症状非特异性,平均延迟22个月出现。全部患者均行腹会阴联合开放入路,广泛切除肿瘤及浸润器官。术前未给予减缩激素治疗。主要观察指标为无病生存期、复发模式和抢救。广泛的盆腔和会阴肿瘤切除是艰难而细致的,但简单易行。(Clavien-Dindo分级(CDC) I级)II)早期和延迟发病。切除范围扩大到子宫切除术、阴道切除术和血管切除术。组织病理及免疫组化示9例侵袭性血管粘液样瘤(AA)、2例纤维瘤病和2例神经纤维瘤(NF)。切除边缘除2(切除边缘1 (R1))外均为充足(R0)。中位随访50个月后,8/13个总系列(61.5%)无病生存。4/9的AA(44%)有局部会阴部和/或盆腔复发(13-37个月),所有患者均可进行补救性切除术;而1/2的纤维瘤病患者死于弥漫性腹膜复发。未见全身转移。广泛的肿瘤,精心的手术,经验丰富的盆腔清扫和切除,可以提供单独的长期治愈,即使在复发后,在一个良好的百分比的小发病率。保留生育能力的切除在肿瘤上并不安全,因为子宫和卵巢经常受到侵犯。
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来源期刊
自引率
25.00%
发文量
58
审稿时长
1 months
期刊介绍: EJGO is dedicated to publishing editorial articles in the Distinguished Expert Series and original research papers, case reports, letters to the Editor, book reviews, and newsletters. The Journal was founded in 1980 the second gynaecologic oncology hyperspecialization Journal in the world. Its aim is the diffusion of scientific, clinical and practical progress, and knowledge in female neoplastic diseases in an interdisciplinary approach among gynaecologists, oncologists, radiotherapists, surgeons, chemotherapists, pathologists, epidemiologists, and so on.
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