Sagar P M, Möslein G, Dozois R R
Division of Colon and Rectal Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
Dis Colon Rectum. 1998 Nov;41(11):1350-5; discussion 1355-6. doi: 10.1007/BF02237046.
The aim of this study was to review the management of desmoid tumors in patients who have undergone ileal pouch-anal anastomosis for familial adenomatous polyposis and their outcome.
A retrospective review of case notes was undertaken in a series of 196 patients with familial adenomatous polyposis who had had an ileal pouch-anal anastomosis. Eleven patients were identified in whom desmoid tumor had developed (4 male; median age, 30 (range, 16-43) years).
Desmoid tumors developed in the abdominal wall alone in 4 of 11 patients. Three enlarging tumors were resected with no subsequent recurrence. No patient received adjuvant treatment and pouch function remained stable at a median follow-up of eight years. Mesenteric desmoid tumors developed in seven patients; the pouch has been excised in two because of ischemia; two other patients have had defunctioning stomas created because of slow progression of their desmoid tumors. One patient had a single-lumen pouch excised and a new S-pouch inserted, with no subsequent recurrence of the desmoid tumor, and in two patients the mesenteric desmoid tumor has remained unchanged. All of these patients received long-term Clinoril (sulindac, Merck & Co., Inc., West Point, PA). Although there has been no significant deterioration in pouch function in patients in whom the pouch remains in situ, the pouch function was significantly worse compared with a control group of patients after ileal pouch-anal anastomosis for familial adenomatous polyposis in whom desmoid tumors did not develop.
Desmoid tumors developed in 5.6 percent of 196 patients after ileal pouch-anal anastomosis for familial adenomatous polyposis. No patient with abdominal-wall desmoid tumor had a recurrence after local excision. By contrast, mesenteric desmoid tumors eventually resulted in pouch failure in four of seven affected patients.
本研究旨在回顾因家族性腺瘤性息肉病接受回肠储袋肛管吻合术患者的硬纤维瘤管理及其结局。
对196例因家族性腺瘤性息肉病接受回肠储袋肛管吻合术的患者病历进行回顾性分析。确定11例发生硬纤维瘤的患者（4例男性；中位年龄30岁（范围16 - 43岁））。
11例患者中4例仅在腹壁发生硬纤维瘤。3例增大的肿瘤切除后未复发。无患者接受辅助治疗，中位随访8年时储袋功能保持稳定。7例患者发生肠系膜硬纤维瘤；2例因缺血切除储袋；另外2例患者因硬纤维瘤进展缓慢行造口转流术。1例患者切除单腔储袋并植入新的S形储袋，硬纤维瘤未复发，2例患者肠系膜硬纤维瘤无变化。所有这些患者均长期服用奇诺力（舒林酸，默克公司，西点，宾夕法尼亚州）。尽管储袋仍保留原位的患者储袋功能无显著恶化，但与家族性腺瘤性息肉病接受回肠储袋肛管吻合术且未发生硬纤维瘤的对照组患者相比，储袋功能明显较差。
196例因家族性腺瘤性息肉病接受回肠储袋肛管吻合术的患者中，5.6%发生硬纤维瘤。腹壁硬纤维瘤患者局部切除后均未复发。相比之下，7例受影响患者中有4例肠系膜硬纤维瘤最终导致储袋功能衰竭。
