Ibrahim Mohanad, Toffaha Ali, Khawar Mahwish, Abdul-Hafez Hamza A, Kurer Mohamed
Department of General Surgery, Hamad Medical Corporation, Doha, Qatar.
Colorectal Surgery Unit, Hamad Medical Corporation, Doha, Qatar.
J Surg Case Rep. 2025 Jul 3;2025(7):rjaf459. doi: 10.1093/jscr/rjaf459. eCollection 2025 Jul.
Desmoid tumors (DT) are rare benign tumors originating from myofibroblasts in the mesentery. Although benign, DTs exhibit local invasion and recurrence and may present sporadically or with familial adenomatous polyposis (FAP) and Gardner's syndrome. The clinical presentation ranges from asymptomatic abdominal masses to severe complications such as bowel obstruction and perforation. This case report highlights a 46-year-old male with acute diffuse peritonitis due to perforation of a mesenteric fibromatosis (MF). Imaging revealed a large mesenteric mass causing bowel perforation, necessitating an urgent exploratory laparotomy. Surgical intervention entailed resection of the affected small bowel and mesentery with temporary stoma formation. Histopathology confirmed MF with negative margins, and the patient was discharged in stable condition. This case highlights the importance of a multidisciplinary approach in managing such complex cases, the challenges in diagnosing and treating intra-abdominal DTs, and the role of surgical resection with careful postoperative monitoring despite recurrence concerns.
韧带样型纤维瘤病(DT)是起源于肠系膜肌成纤维细胞的罕见良性肿瘤。尽管是良性肿瘤,但DT具有局部侵袭性和复发性,可散发出现或与家族性腺瘤性息肉病(FAP)及加德纳综合征相关。临床表现从无症状腹部肿块到肠梗阻和穿孔等严重并发症不等。本病例报告重点介绍了一名46岁男性,因肠系膜纤维瘤病(MF)穿孔导致急性弥漫性腹膜炎。影像学检查发现一个导致肠穿孔的巨大肠系膜肿块,需要紧急进行剖腹探查术。手术干预包括切除受影响的小肠和肠系膜并临时造口。组织病理学证实为MF,切缘阴性,患者出院时病情稳定。本病例强调了多学科方法在处理此类复杂病例中的重要性、诊断和治疗腹腔内DT的挑战,以及尽管存在复发担忧但手术切除并进行仔细术后监测的作用。