Ebeling Peter A, Fun Tristan, Beale Katherine, Cromer Robert, Kempenich Jason W
Surgery, University of Texas Health Science Center at San Antonio, San Antonio, USA.
Surgery, Keesler U.S. Air Force Medical Center, Biloxi, USA.
Cureus. 2019 Jun 17;11(6):e4915. doi: 10.7759/cureus.4915.
Desmoid tumors, also known as aggressive fibromatosis, are fibromuscular neoplasms that arise from mesenchymal cell lines. They may occur in almost all soft tissue compartments. Primary desmoids of the small bowel are rare but potentially serious tumors presenting unique challenges to the general surgeon. We present one case of a 59-year-old man presenting with three months of abdominal distension secondary to a small bowel desmoid. Computed tomography of the abdomen showed an 18-cm mass in the mid-abdomen without obvious vital structure encasement. Percutaneous biopsy of the mass indicated a desmoid tumor. The patient underwent a successful elective exploratory laparotomy with resection and primary enteric anastomosis. Final pathology revealed the mass to be a primary desmoid of the small bowel. His post-operative course was uneventful. At two years after surgery, he is symptom free, and there is no evidence of disease recurrence. Due to the rare nature of primary small bowel desmoids, there are few specific care pathways outlined. This is a challenging pathology to treat that often requires a multidisciplinary team of surgical and medical oncologists.
硬纤维瘤，也称为侵袭性纤维瘤病，是起源于间充质细胞系的纤维肌肉肿瘤。它们几乎可发生于所有软组织间隙。原发性小肠硬纤维瘤罕见但可能是严重的肿瘤，给普通外科医生带来独特挑战。我们报告一例59岁男性患者，因小肠硬纤维瘤导致腹胀3个月。腹部计算机断层扫描显示中腹部有一个18厘米的肿块，无明显重要结构包绕。肿块经皮活检提示为硬纤维瘤。患者成功接受了择期剖腹探查术，进行了切除和一期肠吻合术。最终病理显示肿块为原发性小肠硬纤维瘤。他的术后过程平稳。术后两年，他无症状，无疾病复发迹象。由于原发性小肠硬纤维瘤的罕见性，很少有明确的特定护理路径。这是一种具有挑战性的病理情况，通常需要外科和医学肿瘤学家组成的多学科团队进行治疗。
