Peutz-Jeghers綜合征腸道息肉惡變的臨床分析_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

 費翔 ^1,2 , 于鵬飛 ² , 楊海瑞 ² ,  顧國利 ¹

1. ;
2. ;

Corresponding?author：

顧國利, Email: kzggl@163.com

Keywords：

Peutz-Jeghers綜合征; 黑斑息肉病; 息肉癌變; 診斷; 治療

DOI：

10.7507/1007-9424.20160160

Video：

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目的探討Peutz-Jeghers綜合征腸道息肉惡變的臨床診治經驗。方法對2005～2015年期間所診治的221例Peutz-Jeghers綜合征患者中11例息肉惡變患者的臨床病理資料進行回顧性分析。結果 11例PeutzJeghers綜合征息肉惡變患者確診時的中位年齡為31.36歲。行腸鏡20例次，胃鏡8例次，雙氣囊電子小腸鏡19例次，膠囊內鏡1例次。手術原因依次為腸梗阻、腸套疊、息肉惡變和消化道出血。癌變息肉分別位于十二指腸（4例）、小腸（4例）和結直腸（3例）。病理類型以管狀腺癌（5例）和黏液腺癌（5例）最多見。本組11例患者均無手術死亡，1例術后發生腹部切口感染，經傷口換藥等處置后出院。術后3例患者接受XELOX方案化療，其余未作特殊治療。1例術后失訪，10例隨訪1個月至7年（中位隨訪期2.12年），其中4例術后1～3個月死于十二指腸癌肝肺轉移、空腸腺癌肝轉移和結腸癌肝肺多發轉移，其余患者定期復查消化內鏡或消化道造影等檢查至今。結論 PeutzJeghers綜合征息肉惡變患者年齡較小，早診斷、早治療、定期復查可提高患者的檢出率和臨床治療效果。

Citation： 費翔, 于鵬飛, 楊海瑞, 顧國利. Peutz-Jeghers綜合征腸道息肉惡變的臨床分析. 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, 顧國利, 魏學明, 等. Peutz-Jeghers綜合征臨床綜合治療模式初探(附71例報道).中國普外基礎與臨床雜志, 2012, 19(5):502-506.
2.	全國遺傳性大腸癌協作組.中國人遺傳性大腸癌篩檢標準的實施方案.中華腫瘤雜志, 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.	吳階平, 裘法祖.黃家駟外科學(中冊).第6版.北京:人民衛生出版社, 1999:1109-1110.
7.	蔣曉忠, 陳力恒, 嚴律南, 等. Peutz-Jeghers綜合征及其合并癌變的臨床分析.中國普外基礎與臨床雜志, 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.黑斑息肉綜合征的研究進展.中國普外基礎與臨床雜志, 2005, 12(2):195-198.

1. 王石林, 顧國利, 魏學明, 等. Peutz-Jeghers綜合征臨床綜合治療模式初探(附71例報道).中國普外基礎與臨床雜志, 2012, 19(5):502-506.
2. 全國遺傳性大腸癌協作組.中國人遺傳性大腸癌篩檢標準的實施方案.中華腫瘤雜志, 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. 吳階平, 裘法祖.黃家駟外科學(中冊).第6版.北京:人民衛生出版社, 1999:1109-1110.
7. 蔣曉忠, 陳力恒, 嚴律南, 等. Peutz-Jeghers綜合征及其合并癌變的臨床分析.中國普外基礎與臨床雜志, 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.黑斑息肉綜合征的研究進展.中國普外基礎與臨床雜志, 2005, 12(2):195-198.

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Peutz-Jeghers綜合征腸道息肉惡變的臨床分析

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