中低位直肠癌病理学诊断

直肠是大肠癌最好发的部位,在直肠癌中约70％发生于中低位直肠。在做组织病理学诊断时,需注意以下几点：（1）上皮内瘤病的概念和在与手术治疗的关系,重点介绍了胃肠道上皮瘤形成的Vinna分类和临床处理;（2）组织病理学的预后指标,如手术切缘、直肠外侵犯、局部淋巴结转移、血管和神经束膜的侵犯;（3）大肠癌遗传学,包括遗传性非息肉性结直肠癌和家族性腺瘤性息肉病。     

Gastroduodenal lesions in familial adenomatous polyposis

Prophylactic colectomy is generally recommended for patients with familial adenomatous polyposis (FAP) who are inevitably affected with large bowel cancer. After prophylactic colectomy has been performed, gastrointestinal malignancy is the leading cause of death. Duodenal adenomas are found in patients with FAP and the adenoma–carcinoma sequence exists in the FAP duodenum, suggesting that treatment of duodenal polyps might be beneficial. Several methods of treatment for duodenal lesions in patients with FAP have been reported, but the current treatment options are not ideal. The nonsteroid anti-inflammatory drugs, sulindac and aspirin, are used for chemoprevention, while recently developed cyclooxygenase-2 inhibitors may be of some use in the future. Endoscopic polypectomy has been attempted for duodenal polyps and open surgical polypectomy has proven to be effective for selected patients. Photodynamic therapy and Argon plasma coagulation may be suitable to treat carpeted polyposis. New methods of duodenal resection, such as pancreas-preserving duodenectomy and pylorus-preserving pancreaticoduodenectomy, might be considered for severe duodenal polyposis; however, because prophylactic duodenal surgery has been considered too aggressive, surveillance duodenoscopy is usually performed to detect duodenal cancer at an early stage. Received: June 24, 1999 / Accepted: March 24, 2000     

家族性腺瘤性息肉病的临床分析

目的探讨家族性腺瘤性息肉病（FAP）的早期诊断和治疗方法。方法通过围绕FAP先证者进行家系调查,绘制每个家系的患病分布图,进行系谱分析;回顾性分析资料完整的6个家系,总结其临床特征,对高危亲属进行随访监测。结果 6个家系共83人,受检成员61人;确诊患病20例,发病率32.8%（20/61）。男女比例为13：7,发病平均年龄32.7岁。癌变7例,男：女为4：3,癌变率35%（7/20）,癌变平均年龄42.4岁;7例癌变病人随访期死亡3例,死亡率15%（3/20）。发病20例患者中行全结肠切除术＋直肠部分切除术＋回肠直肠吻合术3例;全结肠切除术＋直肠部分切除术＋回肠直肠吻合术＋回肠贮袋4例,全结肠切除术＋直肠部分切除术＋回肠直肠吻合术＋回肠造瘘术1例,经肛门直肠息肉切除止血术1例,经内镜下高频电切术4例,未作任何治疗7例。行手术治疗患者随访2年,死亡3例,均因肝转移所致。结论通过家系调查,对高危亲属的追踪管理、肠镜监测,可以发现早期病例,行预防性手术效果满意。     

Syndromic gastric polyposis and hereditary gastric cancers

Although the majority of gastric carcinomas are sporadic neoplasms, approximately 10% show familial aggregation, and a hereditary cause is determined in 1–3% cases. Of these, hereditary diffuse gastric cancer (HDGC) is the most recognized predisposition syndrome. Although rare, some of the less commonly reported syndromes (including polyposis syndrome), also confer a markedly increased risk for development of gastric cancer. Identification and characterization of these syndromes requires a multidisciplinary effort involving oncologists, surgeons, genetic counselors, and pathologists.     

Unicryptal gallbladder adenomas in a patient with Gardner's syndrome

Gardner's syndrome occurs when mutation of the adenomatous polyposis coli gene is associated with extra-intestinal manifestations in addition to colorectal adenomas. Only eleven cases of gallbladder adenoma in Gardner's syndrome have been previously reported in the literature. We report a case of Gardner's syndrome in which multiple adenomas are associated with unicryptal adenomas.