Peutz-Jeghers综合征肠道息肉恶变的临床分析_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

 费翔 ^1,2 , 于鹏飞 ² , 杨海瑞 ² ,  顾国利 ¹

1. ;
2. ;

Corresponding author：

顾国利, Email: kzggl@163.com

Keywords：

Peutz-Jeghers综合征; 黑斑息肉病; 息肉癌变; 诊断; 治疗

DOI：

10.7507/1007-9424.20160160

Video：

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目的探讨Peutz-Jeghers综合征肠道息肉恶变的临床诊治经验。方法对2005～2015年期间所诊治的221例Peutz-Jeghers综合征患者中11例息肉恶变患者的临床病理资料进行回顾性分析。结果 11例PeutzJeghers综合征息肉恶变患者确诊时的中位年龄为31.36岁。行肠镜20例次，胃镜8例次，双气囊电子小肠镜19例次，胶囊内镜1例次。手术原因依次为肠梗阻、肠套叠、息肉恶变和消化道出血。癌变息肉分别位于十二指肠（4例）、小肠（4例）和结直肠（3例）。病理类型以管状腺癌（5例）和黏液腺癌（5例）最多见。本组11例患者均无手术死亡，1例术后发生腹部切口感染，经伤口换药等处置后出院。术后3例患者接受XELOX方案化疗，其余未作特殊治疗。1例术后失访，10例随访1个月至7年（中位随访期2.12年），其中4例术后1～3个月死于十二指肠癌肝肺转移、空肠腺癌肝转移和结肠癌肝肺多发转移，其余患者定期复查消化内镜或消化道造影等检查至今。结论 PeutzJeghers综合征息肉恶变患者年龄较小，早诊断、早治疗、定期复查可提高患者的检出率和临床治疗效果。

Citation： 费翔, 于鹏飞, 杨海瑞, 顾国利. Peutz-Jeghers综合征肠道息肉恶变的临床分析. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2016, 23(5): 598-600. doi: 10.7507/1007-9424.20160160 Copy

1.	王石林, 顾国利, 魏学明, 等. Peutz-Jeghers综合征临床综合治疗模式初探(附71例报道).中国普外基础与临床杂志, 2012, 19(5):502-506.
2.	全国遗传性大肠癌协作组.中国人遗传性大肠癌筛检标准的实施方案.中华肿瘤杂志, 2004, 26(3):191-192.
3.	Wang ZJ, Churchman M, Avizienyte E, et al. Germline mutations of the LKB1(STK11) gene in Peutz-Jeghers patients. J Med Genet, 1999, 36(5):365-368.
4.	Smith DP, Rayter SI, Niederlander C, et al. LIP1, a cytoplasmic protein functionally linked to the Peutz-Jeghers syndrome kinase LKB1. Hum Mol Genet, 2001, 10(25):2869-2877.
5.	Jelsig AM, Qvist N, Brusgaard K, et al. Hamartomatous polyposis syndromes:a review. Orphanet J Rare Dis, 2014, 9:101.
6.	吴阶平, 裘法祖.黄家驷外科学(中册).第6版.北京:人民卫生出版社, 1999:1109-1110.
7.	蒋晓忠, 陈力恒, 严律南, 等. Peutz-Jeghers综合征及其合并癌变的临床分析.中国普外基础与临床杂志, 2006, 11(6):709-711.
8.	Giardiello FM, Brensinger JD, Tersmette AC, et al. Very high risk of cancer in familial Peutz-Jeghers syndrome. Gastroenterology, 2000, 119(6):1447-1453.
9.	Giardiello FM, Welsh SB, Hamilton SR, et al. Increased risk of cancer in the Peutz-Jeghers syndrome. N Engl J Med, 1987, 316(24):1511-1514.
10.	Boardman LA, Thibodeau SN, Schaid DJ, et al. Increased risk for cancer in patients with the Peutz-Jeghers syndrome. Ann Intern Med, 1998, 128(11):896-899.
11.	Campos FG, Figueiredo MN, Martinez CA. Colorectal cancer risk in hamartomatous polyposis syndromes. World J Gastrointest Surg, 2015, 7(3):25-32.
12.	Korsse SE, Biermann K, Offerhaus GJ, et al. Identification of molecular alterations in gastrointestinal carcinomas and dysplastic hamartomas in Peutz-Jeghers syndrome. Carcinogenesis, 2013, 34(7):1611-1619.
13.	Song SH, Kim KW, Kim WH, et al. Gastrointestinal cancers in a PeutzJeghers syndrome family:a case report. Clin Endosc, 2013, 46(5):572-575.
14.	Tse JY, Wu S, Shinagare SA, et al. Peutz-Jeghers syndrome:a critical look at colonic Peutz-Jeghers polyps. Mod Pathol, 2013, 26(9):1235-1240.
15.	Dong K, Li B. Peutz-Jeghers syndrome:case reports and update on diagnosis and treatment. Chin J Dig Dis, 2004, 5(4):160-164.
16.	董科, 李波.黑斑息肉综合征的研究进展.中国普外基础与临床杂志, 2005, 12(2):195-198.

1. 王石林, 顾国利, 魏学明, 等. Peutz-Jeghers综合征临床综合治疗模式初探(附71例报道).中国普外基础与临床杂志, 2012, 19(5):502-506.
2. 全国遗传性大肠癌协作组.中国人遗传性大肠癌筛检标准的实施方案.中华肿瘤杂志, 2004, 26(3):191-192.
3. Wang ZJ, Churchman M, Avizienyte E, et al. Germline mutations of the LKB1(STK11) gene in Peutz-Jeghers patients. J Med Genet, 1999, 36(5):365-368.
4. Smith DP, Rayter SI, Niederlander C, et al. LIP1, a cytoplasmic protein functionally linked to the Peutz-Jeghers syndrome kinase LKB1. Hum Mol Genet, 2001, 10(25):2869-2877.
5. Jelsig AM, Qvist N, Brusgaard K, et al. Hamartomatous polyposis syndromes:a review. Orphanet J Rare Dis, 2014, 9:101.
6. 吴阶平, 裘法祖.黄家驷外科学(中册).第6版.北京:人民卫生出版社, 1999:1109-1110.
7. 蒋晓忠, 陈力恒, 严律南, 等. Peutz-Jeghers综合征及其合并癌变的临床分析.中国普外基础与临床杂志, 2006, 11(6):709-711.
8. Giardiello FM, Brensinger JD, Tersmette AC, et al. Very high risk of cancer in familial Peutz-Jeghers syndrome. Gastroenterology, 2000, 119(6):1447-1453.
9. Giardiello FM, Welsh SB, Hamilton SR, et al. Increased risk of cancer in the Peutz-Jeghers syndrome. N Engl J Med, 1987, 316(24):1511-1514.
10. Boardman LA, Thibodeau SN, Schaid DJ, et al. Increased risk for cancer in patients with the Peutz-Jeghers syndrome. Ann Intern Med, 1998, 128(11):896-899.
11. Campos FG, Figueiredo MN, Martinez CA. Colorectal cancer risk in hamartomatous polyposis syndromes. World J Gastrointest Surg, 2015, 7(3):25-32.
12. Korsse SE, Biermann K, Offerhaus GJ, et al. Identification of molecular alterations in gastrointestinal carcinomas and dysplastic hamartomas in Peutz-Jeghers syndrome. Carcinogenesis, 2013, 34(7):1611-1619.
13. Song SH, Kim KW, Kim WH, et al. Gastrointestinal cancers in a PeutzJeghers syndrome family:a case report. Clin Endosc, 2013, 46(5):572-575.
14. Tse JY, Wu S, Shinagare SA, et al. Peutz-Jeghers syndrome:a critical look at colonic Peutz-Jeghers polyps. Mod Pathol, 2013, 26(9):1235-1240.
15. Dong K, Li B. Peutz-Jeghers syndrome:case reports and update on diagnosis and treatment. Chin J Dig Dis, 2004, 5(4):160-164.
16. 董科, 李波.黑斑息肉综合征的研究进展.中国普外基础与临床杂志, 2005, 12(2):195-198.

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Peutz-Jeghers综合征肠道息肉恶变的临床分析

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