Nakada I, Ubukata H, Goto Y, Watanabe Y, Sato S, Tabuchi T, Soma T
Department of Surgery, Tokyo Medical College Kasumigaura Hospital, Ibaraki, Japan.
J Gastroenterol. 1997 Apr;32(2):255-9. doi: 10.1007/BF02936378.
The management of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis (FAP) is very difficult. Non-steroidal anti-inflammatory drugs (NSAIDs), anti-estrogenic agents, and steroids are most commonly used, because surgical removal of these tumors may result in severe morbidity, with local recurrence being common. We report a patient with FAP and intra-abdominal desmoid tumors that regressed markedly after prednisolone therapy. The patient, a 38-year-old woman, had undergone total colectomy and ileorectal anastomosis with a diagnosis of FAP with colon cancer. Approximately 17 months after the surgery, she noticed an elastic firm lump in the abdominal wall. She also experienced lower abdominal distension. Computed tomography (CT) of the lower abdomen showed an invasive heterogenous low-density mass occupying the intra-abdominal space. She was treated with sulindac, NSAID, at 300 mg/day, the diagnosis being intra-abdominal desmoid tumors. She exhibited an intestinal obstruction about 9 months after the initiation of sulindac therapy. We changed the treatment and began prednisolone (initial dose, 40 mg/day). This treatment was continued for two years; subsequently, the lesions regressed markedly. She is currently well, more than 3 years after the withdrawal of prednisolone.
家族性腺瘤性息肉病（FAP）患者腹腔内硬纤维瘤的治疗非常困难。非甾体类抗炎药（NSAIDs）、抗雌激素药物和类固醇是最常用的，因为手术切除这些肿瘤可能导致严重的并发症，局部复发很常见。我们报告一例FAP合并腹腔内硬纤维瘤的患者，其在泼尼松龙治疗后肿瘤明显消退。该患者为一名38岁女性，因FAP合并结肠癌接受了全结肠切除术和回肠直肠吻合术。手术后约17个月，她注意到腹壁有一个弹性坚实的肿块。她还出现了下腹胀。下腹部计算机断层扫描（CT）显示一个侵入性的不均匀低密度肿块占据腹腔空间。她接受舒林酸（一种NSAID）治疗，剂量为每日300毫克，诊断为腹腔内硬纤维瘤。在舒林酸治疗开始约9个月后，她出现了肠梗阻。我们改变了治疗方案，开始使用泼尼松龙（初始剂量为每日40毫克）。该治疗持续了两年；随后，病变明显消退。在停用泼尼松龙3年多后，她目前状况良好。
