Hajri Mohamed, Talbi Ghofrane, Ferjaoui Wael, Atallah Aziz, Ben Slama Sana, Mestiri Hafedh, Bayar Rached
Department of General Surgery, Mongi Slim University Hospital, Tunis, Tunisia.
Faculty of Medicine of Tunis, Tunis El Manar University, Tunis, Tunisia.
Ann Med Surg (Lond). 2022 May 10;78:103741. doi: 10.1016/j.amsu.2022.103741. eCollection 2022 Jun.
Desmoid-type fibromatosis, also known as desmoid tumors, are rare fibroblastic neoplasms that account for less than 3% of all soft tissue tumors. Although they are benign neoplasms without metastatic potential, they are known to be locally aggressive and may invade adjacent structures leading to fatal complications.
We describe the case of a 26-year-old woman who presenting with the clinical picture of acute peritonitis. Emergency surgery was performed and a large poorly-circumscribed heterogeneous tumor was found, occupying the jejunum mesentery and infiltrating the jejunal wall causing its perforation into the abdominal cavity. En bloc resection of the tumor and the involved jejunum was performed. Histology and immunohistochemistry confirmed it to be mesenteric desmoid-type fibromatosis. The postoperative course was uneventful and the patient had no evidence of recurrence 18 months after tumor resection.
Mesenteric desmoid-type fibromatosis is a rare condition with insidious growth and locally aggressive behavior. Serious complications such as bowel perforation are rare but possible, as shown in our presentation. Complete surgical resection is the first-line treatment bur high recurrence rates remain problematic.
韧带样型纤维瘤病，也称为韧带样瘤，是一种罕见的成纤维细胞肿瘤，占所有软组织肿瘤的比例不到3%。尽管它们是无转移潜能的良性肿瘤，但已知具有局部侵袭性，可能侵犯相邻结构，导致致命并发症。
我们描述了一名26岁女性的病例，她表现出急性腹膜炎的临床症状。进行了急诊手术，发现一个边界不清的大的异质性肿瘤，占据空肠系膜并浸润空肠壁，导致其穿孔进入腹腔。对肿瘤和受累空肠进行了整块切除。组织学和免疫组化证实为肠系膜韧带样型纤维瘤病。术后过程顺利，肿瘤切除18个月后患者无复发迹象。
肠系膜韧带样型纤维瘤病是一种罕见疾病，生长隐匿且具有局部侵袭性。如我们的病例所示，肠穿孔等严重并发症虽罕见但有可能发生。完整的手术切除是一线治疗方法，但高复发率仍然是个问题。
