Mitchem Jonathan B, Hall Jason F
Department of Colon and Rectal Surgery, Lahey Hospital and Medical Center, Burlington, Massachusetts.
Clin Colon Rectal Surg. 2016 Dec;29(4):321-329. doi: 10.1055/s-0036-1584089.
Familial adenomatous polyposis (FAP) syndromes make up fewer than 1% of patients diagnosed with colorectal cancer each year. Patients with familial polyposis syndromes including FAP, attenuated FAP, and MYH-associated polyposis (MAP), are an important group often cared for by colorectal surgeons. Registry and screening programs have been shown to improve survival in patients with adenomatous polyposis, as it allows patients to undergo surgical intervention prior to the development of colorectal cancer. There are several surgical options for the treatment of colorectal polyps in patients with adenomatous polyposis, so it is important to choose the appropriate procedure for each patient after discussing the risk of cancer in the rectal remnant, as well as bowel and sexual function in a predominantly young patient group. Regardless of procedure choice, long-term follow-up is important with yearly endoscopic evaluation of the pouch or remnant rectum, as well as appropriate screening for extracolonic malignancy. Adenomatous polyposis patients require an intense care regimen, but can have a normal lifespan with good quality when cared for appropriately.
家族性腺瘤性息肉病(FAP)综合征患者每年占确诊为结直肠癌患者的比例不到1%。患有家族性息肉病综合征(包括FAP、轻型FAP和MYH相关性息肉病(MAP))的患者是一个重要群体,常由结直肠外科医生诊治。登记和筛查项目已被证明可提高腺瘤性息肉病患者的生存率,因为这能让患者在结直肠癌发生之前接受手术干预。对于患有腺瘤性息肉病的患者,有几种手术方法可用于治疗结直肠息肉,因此在讨论直肠残端的癌症风险以及主要为年轻患者群体的肠道和性功能后,为每位患者选择合适的手术程序很重要。无论手术方式如何选择,长期随访都很重要,每年要对储袋或残余直肠进行内镜评估,并对结肠外恶性肿瘤进行适当筛查。腺瘤性息肉病患者需要严格的护理方案,但如果得到适当护理,可拥有正常寿命且生活质量良好。