Tytgat G N, Gopinath N
Department of Gastroenterology, Academic Medical Center, Amsterdam, The Netherlands.
Eur J Cancer. 1995 Jul-Aug;31A(7-8):1154-9. doi: 10.1016/0959-8049(95)00154-b.
Most small bowel polyps in familial adenomatous polyposis (FAP) occur in the peri-ampullary region, and distal small bowel adenomas and carcinomas are comparatively less common. As standard therapy in FAP consists of proctocolectomy with ileal pouch anal anastomosis, or ileorectal anastomosis, it is essential to be aware of the potential for adenomatous polyp formation in the terminal ileum and rectum. Ileal adenomas are found in 9-20% of patients with FAP, and new polyps may develop after colectomy. Ileal lymphoid hyperplasia and polyps are 2-4 times more common than adenomas, may be indistinguishable from adenomas on examination (requiring biopsy for diagnosis), and tend to regress after colectomy. Adenomas may arise in pouches, usually after an interval of several years, and have been documented to occur in the terminal ileum up to 25 years after colectomy. At pouch construction, rectal mucosectomy may theoretically fail to remove all mucosa at risk. Small islets of rectal mucosa may remain after this technically difficult operation, and the late development of cancer, up to 20 years postoperation has been noted. A stapled anastomosis may arguably have a better physiological result, but a greater amount of residual rectal mucosa may increase late cancer risk. Annual endoscopic follow-up of pouches is recommended. All polyps or suspicious lesions should be biopsied, excised or destroyed, preserving a sample for histology. After ileorectal anastomosis, cancer risk in the rectal stump increases with chronological age, with risk ranging from 5-10% at age 50 years, to 14-29% at age 60 years. Surveillance of the rectal stump in FAP is recommended every 4-6 months. There may be a role for prostaglandin synthesis inhibitors in some patients.
家族性腺瘤性息肉病(FAP)中的大多数小肠息肉发生在壶腹周围区域,而远端小肠腺瘤和癌相对较少见。由于FAP的标准治疗包括直肠结肠切除术加回肠袋肛管吻合术或回肠直肠吻合术,因此必须意识到回肠末端和直肠发生腺瘤性息肉的可能性。在9%-20%的FAP患者中可发现回肠腺瘤,结肠切除术后可能会出现新的息肉。回肠淋巴组织增生和息肉比腺瘤常见2-4倍,在检查时可能与腺瘤难以区分(需要活检以确诊),并且在结肠切除术后往往会消退。腺瘤可能出现在袋囊中,通常在数年的间隔后出现,并且有记录显示在结肠切除术后长达25年回肠末端仍会发生腺瘤。在构建袋囊时,理论上直肠黏膜切除术可能无法切除所有有风险的黏膜。在这项技术难度较大的手术后,可能会残留小片状直肠黏膜,并且已注意到术后长达20年发生癌症的情况。吻合器吻合术可能在生理结果方面更好,但更多的残留直肠黏膜可能会增加晚期癌症风险。建议对袋囊进行每年一次的内镜随访。所有息肉或可疑病变均应进行活检、切除或破坏,并保留样本用于组织学检查。回肠直肠吻合术后,直肠残端的癌症风险随年龄增长而增加,50岁时风险为5%-10%,60岁时风险为14%-29%。建议对FAP患者的直肠残端每4-6个月进行一次监测。前列腺素合成抑制剂在一些患者中可能会发挥作用。