Knudsen A L, Bülow S
H:S Hvidovre Hospital, kirurgisk sektion.
Ugeskr Laeger. 2000 Oct 16;162(42):5628-31.
Desmoid tumors (DT) are rare benign tumors that do not metastasize, but tend to invade locally. DT are frequently seen in patients with familial adenomatous polyposis (FAP), and diagnosis and treatment are often difficult.
The article presents the clinical picture, diagnosis and treatment of DT in patients registered in the Danish Polyposis Register by the end of 1999.
Twenty-seven of 486 patients (6%) had DT. Eighteen patients were alive at the time of evaluation. DT were found in the mesentery in 42%, in the abdominal wall in 40%, in the retroperitoneum in 8% and only 10% on the extremities. Fifty percent of the patients had complications (intestinal obstruction, hydronephrosis or fistulas), and 2/9 deaths were caused by DT. Ninety-three percent were treated with surgery, NSAIDs, antioestogenic drugs, chemotherapy or radiotherapy, but all modalities proved disappointing, except for treatment with a combination of the NSAID sulindac and tamoxifen. Five patients treated with this combination showed extensive and long lasting response.
Surgical excision is recommended in patients with DT in the abdominal wall. First line treatment of mesenteric DT is Clinoril in combination with tamoxifen. Elective surgery may be considered in patients with a small well-defined DT with no signs of invasion of vital structures, and in patients with imminent bowel ischaemia or obstruction. The prognosis for mesenteric DT is grave, and improvement of the therapeutic strategy awaits current international studies.
硬纤维瘤（DT）是一种罕见的良性肿瘤，不会发生转移，但易于局部侵袭。硬纤维瘤常见于家族性腺瘤性息肉病（FAP）患者，其诊断和治疗往往具有挑战性。
本文介绍了截至1999年底在丹麦息肉病登记处登记的硬纤维瘤患者的临床表现、诊断和治疗情况。
486例患者中有27例（6%）患有硬纤维瘤。评估时18例患者存活。硬纤维瘤在肠系膜中占42%，腹壁中占40%，腹膜后占8%，四肢仅占10%。50%的患者出现并发症（肠梗阻、肾积水或瘘管），9例死亡中有2例由硬纤维瘤导致。93%的患者接受了手术、非甾体抗炎药、抗雌激素药物、化疗或放疗，但除了非甾体抗炎药舒林酸和他莫昔芬联合治疗外，所有治疗方式都令人失望。5例接受该联合治疗的患者显示出广泛且持久的反应。
腹壁硬纤维瘤患者建议手术切除。肠系膜硬纤维瘤的一线治疗是舒林酸联合他莫昔芬。对于小的边界清晰且无重要结构侵犯迹象的硬纤维瘤患者，以及即将出现肠缺血或肠梗阻的患者，可考虑择期手术。肠系膜硬纤维瘤的预后严重，治疗策略的改善有待当前的国际研究。
