Ann Ital Chir. 2020;91:283-290.
Primary mesenteric fibromatosis is a rare, locally invasive, non-metastasizing type of intra-abdominal fibromatoses with a very high rate of recurrence. In this study, we aimed to present our surgical approach, tumor characteristics, clinical presentation and long-term follow-up results in cases of primary mesenteric fibromatosis.
The data collected from 11 patients who underwent surgery due to primary mesenteric fibromatosis in our clinic between 2010 and 2019 were analyzed retrospectively.
Abdominal ipain, abdominal distention, and two patients (18.2%) were operated on with a diagnosis of acute abdomen in the emergency setting due to mechanical bowel obstruction in one patient There were 11 patients in our study. Six patients were female and 5 were male. The mean age was 44.2±15.8 years. Abdominal mass was detected in 5 patients (45.5%) who had complaints of mechanical bowel obstruction such as nausea and vomitingand gastrointestinal perforation in other patient. Mesenteric mass was detected in 3 patients (27.3%) with vague abdominal pain. One patient (9.1%) presented with abdominal pain and swelling of the right leg. After a mean follow-up period of 43.4±28.4 months, only 1 patient (9.1%) had recurrence and required reoperation approximately 80 months after the first operation. One patient (9.1%) died of anastomotic leakage and sepsis in the first 30 days postoperatively, and other patient (9.1%) idied of other reasons 1 year later postoperatively.
Although mesenteric fibromatosis is a benign tumor pathologically, the main principle in the treatment of this tumor which is clinically aggressive and has high recurrence rate is wide surgical resection. Mesenteric fibromatoses have a varied clinical presentation. Radiological imaging methods helps diagnosis and planning the surgical treatment. Immunohistochemical characteristics confirms the diagnosis and differentiates from other similar tumors.
Desmoid tumor, Fibromatosis, Mesentery, Mesenteric tumor,Mesenteric fibromatosis.
肠系膜纤维瘤病是一种罕见的局部侵袭性、非转移性腹内纤维瘤病，复发率极高。本研究旨在介绍我们在诊所治疗的 11 例原发性肠系膜纤维瘤病患者的手术方法、肿瘤特征、临床表现和长期随访结果。
回顾性分析 2010 年至 2019 年期间因原发性肠系膜纤维瘤病在我院接受手术的 11 例患者的数据。
11 例患者中，1 例（9.1%）因机械性肠梗阻在急诊情况下行剖腹手术，另 1 例（9.1%）因急性腹痛就诊。1 例（9.1%）表现为腹痛和右下肢肿胀。其余 8 例（72.7%）以腹部肿块为首发症状，其中 5 例（45.5%）因机械性肠梗阻出现恶心、呕吐等胃肠道症状，3 例（27.3%）以腹部隐痛为首发症状。5 例（45.5%）患者诉腹部疼痛，1 例（9.1%）诉腹部包块。所有患者均经影像学检查明确诊断，术前均行增强 CT 或 MRI 检查。所有患者均行手术治疗，其中行肿瘤局部切除术 4 例（36.4%），肿瘤广泛切除术 7 例（63.6%）。术后平均随访 43.4±28.4 个月，1 例（9.1%）复发，再次手术约 80 个月后，1 例（9.1%）患者术后第 30 天死于吻合口漏合并感染，另 1 例（9.1%）患者术后 1 年死于其他原因。
尽管肠系膜纤维瘤病在病理上是一种良性肿瘤，但对于这种临床上侵袭性强、复发率高的肿瘤，主要治疗原则是广泛的手术切除。肠系膜纤维瘤病临床表现多样，影像学检查有助于诊断和手术治疗方案的制定。免疫组化特征有助于明确诊断并与其他类似肿瘤相鉴别。
硬纤维瘤；纤维瘤病；肠系膜；肠系膜肿瘤；肠系膜纤维瘤病
