Church J M, McGannon E
David G. Jagelman Inherited Colorectal Cancer Registries, Department of Colorectal Surgery, Cleveland Clinic Foundation, Ohio, USA.
Dis Colon Rectum. 2000 Apr;43(4):445-50. doi: 10.1007/BF02237185.
Intra-abdominal desmoid tumors occur in 12 percent of patients with familial adenomatous polyposis. A minority grow quickly and are lethal, most are relatively inert, and some cause problems by obstructing adjacent organs. Desmoid tumors may be estrogen-dependent, and estrogen-blocking drugs are part of the usual treatment of these tumors. This study was performed to examine the effect of pregnancy on the course of patients with familial adenomatous polyposis and intra-abdominal desmoids.
All females with familial adenomatous polyposis and an intra-abdominal desmoid treated or followed up at this institution were eligible. Stable, asymptomatic desmoids were followed up yearly with examination and CT scan. Growing or symptomatic desmoids were followed up at least every six months. Maximum tumor size was grouped as follows: <10 cm, 10 to 20 cm, and >20 cm. A change in tumor size was defined as a change of +/-50 percent or more of maximum diameter. Stable tumors showed no change in diameter during the study period; variable growth was defined as a significant change in either direction that was followed by a return to previous dimensions or a stabilization of growth. Rapid growth was a doubling of diameter within three months. Pregnant females were compared with nonpregnant females. Subgroups of females were matched for age at diagnosis of desmoid.
Twenty-two females had never been pregnant, whereas 25 had been pregnant at least once. Eleven pairs were matched for age. There were no differences between groups in the incidence of extracolonic manifestations of familial adenomatous polyposis, family history of desmoids, number or type of surgeries done for familial adenomatous polyposis, length of follow-up, or time from surgery to desmoid diagnosis. Desmoids in pregnant females had a significantly more benign course: 18 were stable (vs. 6 nonpregnant females), 2 had variable growth (vs. 10), 1 had rapid growth (vs. 5), and 4 disappeared (vs. 1). There were also trends to smaller, less symptomatic tumors requiring treatment less often in pregnant females.
Pregnancy seems to ameliorate the course of abdominal desmoid tumors significantly in females with familial adenomatous polyposis. This finding raises questions about the most appropriate hormonal treatment for these tumors. Perhaps progesterone or prolactin therapy should be tried, alone or in combination with estrogen. If further studies confirm these findings, females with a family history of desmoid tumors should not be advised against pregnancy.
腹内硬纤维瘤在家族性腺瘤性息肉病患者中的发生率为12%。少数硬纤维瘤生长迅速且致命,多数相对惰性,有些因阻塞相邻器官而引发问题。硬纤维瘤可能依赖雌激素,雌激素阻断药物是这些肿瘤常规治疗的一部分。本研究旨在探讨妊娠对家族性腺瘤性息肉病合并腹内硬纤维瘤患者病程的影响。
所有在本机构接受治疗或随访的患有家族性腺瘤性息肉病及腹内硬纤维瘤的女性均符合条件。稳定、无症状的硬纤维瘤每年进行检查和CT扫描随访。生长性或有症状的硬纤维瘤至少每六个月随访一次。最大肿瘤大小分为以下几组:<10 cm、10至20 cm和>20 cm。肿瘤大小的变化定义为最大直径变化±50%或更多。稳定的肿瘤在研究期间直径无变化;可变生长定义为直径在任一方向上有显著变化,随后恢复到先前尺寸或生长稳定。快速生长是指在三个月内直径翻倍。将怀孕女性与未怀孕女性进行比较。对硬纤维瘤诊断时年龄匹配的女性亚组进行匹配。
22名女性从未怀孕,而25名女性至少怀孕过一次。11对年龄匹配。两组在家族性腺瘤性息肉病的结肠外表现发生率、硬纤维瘤家族史、为家族性腺瘤性息肉病进行的手术数量或类型、随访时间或从手术到硬纤维瘤诊断的时间方面无差异。怀孕女性的硬纤维瘤病程明显更良性:18例稳定(未怀孕女性为6例),2例可变生长(未怀孕女性为10例),1例快速生长(未怀孕女性为5例),4例消失(未怀孕女性为1例)。怀孕女性的肿瘤也有更小、症状更少且需要治疗次数更少的趋势。
妊娠似乎能显著改善家族性腺瘤性息肉病女性腹内硬纤维瘤的病程。这一发现引发了关于这些肿瘤最合适激素治疗的问题。或许应尝试单独或联合雌激素使用孕激素或催乳素治疗。如果进一步研究证实这些发现,不应建议有硬纤维瘤家族史的女性避免怀孕。