Penna C, Tiret E, Parc R, Sfairi A, Kartheuser A, Hannoun L, Nordlinger B
Department of Alimentary Tract Surgery, Hôpital Saint-Antoine, Paris, France.
Surg Gynecol Obstet. 1993 Sep;177(3):263-8.
Abdominal desmoid tumors (ADT) have become an important problem because of the increased survival of patients with familial adenomatous polyposis (FAP). Of 240 patients operated upon for FAP between 1978 and 1991, 29 (16 men and 13 women) had ADT. Diagnosis was made at laparotomy in 19 patients, while an abdominal mass or intestinal obstruction was the principal cause of discovery in the ten remaining patients. Twenty ADT were discovered after a previous laparotomy performed an average of three years earlier. Nine ADT occurred in 146 patients who had previously undergone a coloproctectomy and ileal pouch-anal anastomosis and eight occurred in 74 patients who had abdominal colectomy and ileorectal anastomosis. Desmoid tumors were responsible for eight instances of intestinal obstruction and one instance of small intestinal perforation. One death was directly related to ADT. In four of nine patients, ADT was responsible for a deterioration of the functional results of ileoanal anastomosis, but pouch removal was not necessary. Conversion of ileorectal anastomosis to ileoanal anastomosis and excision of carcinomas that occur in the residual rectum after ileorectal anastomosis were impossible because of ADT in three and two patients, respectively. Complete surgical removal of the tumor was possible in seven patients only and four patients had a recurrence. None of the medical therapies used (sulindac, tamoxifen and chemotherapy) were effective. The results of this study confirm the high incidence, severity and absence of effective treatment of ADT in FAP. Desmoid tumors occurring after ileorectal anastomosis seem to be more severe than after ileoanal anastomosis because, in the former instance, they may interfere with the further management of the rectal stump.
由于家族性腺瘤性息肉病（FAP）患者生存率的提高，腹壁硬纤维瘤（ADT）已成为一个重要问题。在1978年至1991年间接受FAP手术的240例患者中，有29例（16例男性和13例女性）患有ADT。19例患者在剖腹手术时确诊，其余10例患者则以腹部肿块或肠梗阻为主要发现原因。20例ADT是在平均三年前进行的一次剖腹手术后发现的。9例ADT发生在146例先前接受结肠直肠切除术和回肠储袋肛管吻合术的患者中，8例发生在74例接受腹部结肠切除术和回肠直肠吻合术的患者中。硬纤维瘤导致8例肠梗阻和1例小肠穿孔。1例死亡与ADT直接相关。在9例患者中的4例中，ADT导致回肠肛管吻合术的功能结果恶化，但无需切除储袋。分别有3例和2例患者因ADT而无法将回肠直肠吻合术转换为回肠肛管吻合术以及切除回肠直肠吻合术后残留直肠中发生的癌。仅7例患者能够完全手术切除肿瘤，4例患者复发。所使用的任何药物治疗（舒林酸、他莫昔芬和化疗）均无效。本研究结果证实了FAP中ADT的高发病率、严重性以及缺乏有效治疗方法。回肠直肠吻合术后发生的硬纤维瘤似乎比回肠肛管吻合术后更严重，因为在前一种情况下，它们可能会干扰直肠残端的进一步处理。
