为了使梗阻、出血或肿瘤等并发症发生的风险降到最低,推荐患者进行监测。 每年都需要回顾病史和体检。 应根据胃肠道肿瘤的风险来制定内镜监测的方案,这取决于内镜下息肉的外观、数目、大小以及一些诸如年龄和家族史等因素。 也要监测肠道外肿瘤,最好由一个多学科团队进行监测。 最好在多学科综合团队的护理下进行监测。 这个团队可能包括胃肠病学家、结直肠外科医师、乳腺科专家、妇科医师、遗传学家、遗传咨询师和基层医疗人员。
应在 8 岁时进行食管胃十二指肠镜检查 (oesophagogastroduodenoscopy, OGD) 和结肠镜检查。[15]Syngal S, Brand RE, Church JM, et al; American College of Gastroenterology. ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110(2):223-62.http://gi.org/wp-content/uploads/2015/02/ACG_Guideline_Hereditary-Gastrointestinal-Cancer-Syndromes_February_2015.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/25645574?tool=bestpractice.com[16]Beggs AD, Latchford AR, Vasen HF, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010 Jul;59(7):975-86.http://www.ncbi.nlm.nih.gov/pubmed/20581245?tool=bestpractice.com如果发现息肉,这两种检查应每 3 年进行一次;如果没有发现息肉,随后应在 18 岁时进行一次基线检查,之后每 3 年进行一次检查。[15]Syngal S, Brand RE, Church JM, et al; American College of Gastroenterology. ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110(2):223-62.http://gi.org/wp-content/uploads/2015/02/ACG_Guideline_Hereditary-Gastrointestinal-Cancer-Syndromes_February_2015.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/25645574?tool=bestpractice.com[16]Beggs AD, Latchford AR, Vasen HF, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010 Jul;59(7):975-86.http://www.ncbi.nlm.nih.gov/pubmed/20581245?tool=bestpractice.com
乳腺监测(女性):在 18 岁开始进行自我乳腺检查,在 25 岁开始每年进行一次乳腺核磁共振成像 (magnetic resonance imaging, MRI),在 50 岁之后,转为进行 X 线乳腺摄影。[15]Syngal S, Brand RE, Church JM, et al; American College of Gastroenterology. ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110(2):223-62.http://gi.org/wp-content/uploads/2015/02/ACG_Guideline_Hereditary-Gastrointestinal-Cancer-Syndromes_February_2015.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/25645574?tool=bestpractice.com[16]Beggs AD, Latchford AR, Vasen HF, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010 Jul;59(7):975-86.http://www.ncbi.nlm.nih.gov/pubmed/20581245?tool=bestpractice.com
胰腺监测:推荐超声内镜和/或MRI/磁共振胰胆管造影。 国际胰腺癌筛选同盟关于合适的筛选方式和随访间期并没有给出一个明确的推荐,但是既往的专家意见建议从25-30岁开始每1-2年进行一次筛查,和/或行CA19-9检测。[15]Syngal S, Brand RE, Church JM, et al; American College of Gastroenterology. ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110(2):223-62.http://gi.org/wp-content/uploads/2015/02/ACG_Guideline_Hereditary-Gastrointestinal-Cancer-Syndromes_February_2015.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/25645574?tool=bestpractice.com[16]Beggs AD, Latchford AR, Vasen HF, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010 Jul;59(7):975-86.http://www.ncbi.nlm.nih.gov/pubmed/20581245?tool=bestpractice.com
妇产科监测:在 25 岁开始每年进行一次盆腔检查;在 25 岁开始进行经阴道超声检查。[15]Syngal S, Brand RE, Church JM, et al; American College of Gastroenterology. ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110(2):223-62.http://gi.org/wp-content/uploads/2015/02/ACG_Guideline_Hereditary-Gastrointestinal-Cancer-Syndromes_February_2015.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/25645574?tool=bestpractice.com专家意见建议从 25 岁开始定期进行宫颈筛查,包括采用液基细胞学检查的宫颈涂片,每 2-3 年进行一次。[16]Beggs AD, Latchford AR, Vasen HF, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010 Jul;59(7):975-86.http://www.ncbi.nlm.nih.gov/pubmed/20581245?tool=bestpractice.com
睾丸监测(男性):从出生至12岁,每年都需要注意对睾丸进行体格检查。[15]Syngal S, Brand RE, Church JM, et al; American College of Gastroenterology. ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110(2):223-62.http://gi.org/wp-content/uploads/2015/02/ACG_Guideline_Hereditary-Gastrointestinal-Cancer-Syndromes_February_2015.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/25645574?tool=bestpractice.com如果发现异常,需通过睾丸超声进行随访。[16]Beggs AD, Latchford AR, Vasen HF, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010 Jul;59(7):975-86.http://www.ncbi.nlm.nih.gov/pubmed/20581245?tool=bestpractice.com青少年年龄段之后,不常规进行监测,可根据个体情况考虑是否进行监测。[15]Syngal S, Brand RE, Church JM, et al; American College of Gastroenterology. ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110(2):223-62.http://gi.org/wp-content/uploads/2015/02/ACG_Guideline_Hereditary-Gastrointestinal-Cancer-Syndromes_February_2015.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/25645574?tool=bestpractice.com
对于吸烟者,应考虑每年进行放射影像学检查或胸部 CT 扫描。[15]Syngal S, Brand RE, Church JM, et al; American College of Gastroenterology. ACG clinical guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015 Feb;110(2):223-62.http://gi.org/wp-content/uploads/2015/02/ACG_Guideline_Hereditary-Gastrointestinal-Cancer-Syndromes_February_2015.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/25645574?tool=bestpractice.com关于症状和戒烟的教育。