纤维瘤是起源于纤维-腱膜组织的梭形细胞的良性增殖。许多家族性腺瘤性息肉病(FAP)患者死于硬纤维瘤,可以自发产生,但通常似乎是由预防性结肠切除术手术诱导的。纤维瘤是FAP患者死亡的第二大常见原因,仅次于结直肠癌。许多患者可以在没有症状的情况下长寿,但是当出现症状(从肠或输尿管梗阻到肠穿孔伴脓肿和瘘管)或存在功能损害的风险时,广泛的疗法(局部和全身)在改善症状和控制疾病方面是有价值的。半个日本人,半白人男性,在13岁时被诊断出患有与FAP相关的腹内硬纤维瘤的患者,从38岁开始接受腹壁切口减压和化疗治疗.治疗结果是进行性疾病,基于改良的实体瘤反应评估标准(mRECIST),当他41岁到我们医院就诊时,硬纤维瘤侵入小肠,腹壁有瘘。我们做了一个姑息手术来改善他的症状,发烧,腹痛,呕吐,和进食困难。由于肿瘤非常大,侵入了小肠,需要包括小肠在内的大量切除.为预期的大出血做准备,手术前将球囊导管置入肠系膜上动脉.虽然手术非常困难,手术后,患者恢复了进食能力,出院时可以走动,没有短肠综合征。我们报告了我们治疗最大的腹膜内硬纤维瘤之一的经验。手术切除后病程良好,改善生活质量和预后。
Desmoid tumors are benign proliferations of spindle cells originating in fibro-aponeurotic tissue. Many patients with familial adenomatous polyposis (FAP) die from desmoid tumors, which can arise spontaneously but often appear to be surgically induced by prophylactic colectomy. Desmoid tumors are the second most common cause of death in patients with FAP, second to colorectal cancer. Many patients can live a long life with desmoid tumors without symptoms, but when symptoms (ranging from bowel or ureteric obstruction to bowel perforation with abscess and fistula) appear or there is a risk of functional impairment, a wide spectrum of therapies (local and systemic) are valuable in improving the symptoms and controlling the disease. A half-Japanese, half-Caucasian male, who had been diagnosed with intra-abdominal desmoid tumors associated with FAP at age 13, was treated using abdominal wall incision for decompression and chemotherapy from the age of 38. The therapeutic outcome was progressive disease, based on the modified response evaluation criteria in solid tumors (mRECIST), and when he visited our hospital at age 41 the desmoid tumor had invaded the small bowel with a fistula to the abdominal wall. We performed a palliative operation to improve his symptoms, which were fever, abdominal pain, vomiting, and difficulty eating. As the tumor was extremely large and had invaded the small intestine, massive resection including the small intestine was required. To prepare for anticipated massive bleeding, a balloon catheter was placed in the superior mesenteric artery just prior to surgery. Although the operation was extremely difficult, following surgery the patient regained his ability to eat and when discharged was ambulatory and without short-bowel syndrome. We report our experience treating one of the largest reported intraperitoneal desmoid tumors. Resection resulted in a good postoperative course, with improved quality of life and prognosis.