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Peutz-Jeghers Polyp of the Small Intestine

MedGen UID:
78552
•Concept ID:
C0265323
•
Neoplastic Process
Synonyms: Peutz-Jeghers Hamartoma of Small Bowel; Peutz-Jeghers Hamartoma of Small Intestine; Peutz-Jeghers Hamartoma of the Small Bowel; Peutz-Jeghers Hamartoma of the Small Intestine; Peutz-Jeghers Harmatoma of Small Intestine; Peutz-Jeghers Harmatoma of the Small Intestine; Peutz-Jeghers Polyp of Small Intestine; Peutz-Jeghers polyps of small bowel; Peutz-Jeghers Small Bowel Hamartoma; Peutz-Jeghers Small Intestine Hamartoma
SNOMED CT: Peutz-Jeghers polyps of small bowel (53633000)

Definition

A non-neoplastic hamartomatous polyp that arises from the small intestine. It is characterized by the presence of smooth muscle branching bands, and cystic mucosal changes. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPeutz-Jeghers Polyp of the Small Intestine

Professional guidelines

PubMed

Updates in the diagnosis and management of non-ampullary small-bowel polyposis.
Lucaciu L, Yano T, Saurin JC
Best Pract Res Clin Gastroenterol 2023 Jun-Aug;64-65:101852. Epub 2023 Jul 22 doi: 10.1016/j.bpg.2023.101852. PMID: 37652652
Clinical Guidelines for Diagnosis and Management of Peutz-Jeghers Syndrome in Children and Adults.
Yamamoto H, Sakamoto H, Kumagai H, Abe T, Ishiguro S, Uchida K, Kawasaki Y, Saida Y, Sano Y, Takeuchi Y, Tajika M, Nakajima T, Banno K, Funasaka Y, Hori S, Yamaguchi T, Yoshida T, Ishikawa H, Iwama T, Okazaki Y, Saito Y, Matsuura N, Mutoh M, Tomita N, Akiyama T, Yamamoto T, Ishida H, Nakayama Y
Digestion 2023;104(5):335-347. Epub 2023 Apr 13 doi: 10.1159/000529799. PMID: 37054692
Management of small bowel polyps: A literature review.
de Latour RA, Kilaru SM, Gross SA
Best Pract Res Clin Gastroenterol 2017 Aug;31(4):401-408. Epub 2017 Jun 27 doi: 10.1016/j.bpg.2017.06.003. PMID: 28842049

Recent clinical studies

Etiology

Clinical Guidelines for Diagnosis and Management of Peutz-Jeghers Syndrome in Children and Adults.
Yamamoto H, Sakamoto H, Kumagai H, Abe T, Ishiguro S, Uchida K, Kawasaki Y, Saida Y, Sano Y, Takeuchi Y, Tajika M, Nakajima T, Banno K, Funasaka Y, Hori S, Yamaguchi T, Yoshida T, Ishikawa H, Iwama T, Okazaki Y, Saito Y, Matsuura N, Mutoh M, Tomita N, Akiyama T, Yamamoto T, Ishida H, Nakayama Y
Digestion 2023;104(5):335-347. Epub 2023 Apr 13 doi: 10.1159/000529799. PMID: 37054692
Peutz-Jeghers syndrome.
Tacheci I, Kopacova M, Bures J
Curr Opin Gastroenterol 2021 May 1;37(3):245-254. doi: 10.1097/MOG.0000000000000718. PMID: 33591027
Small Bowel Neoplasms and Polyps.
Pourmand K, Itzkowitz SH
Curr Gastroenterol Rep 2016 May;18(5):23. doi: 10.1007/s11894-016-0497-x. PMID: 27086004
Update on imaging of Peutz-Jeghers syndrome.
Tomas C, Soyer P, Dohan A, Dray X, Boudiaf M, Hoeffel C
World J Gastroenterol 2014 Aug 21;20(31):10864-75. doi: 10.3748/wjg.v20.i31.10864. PMID: 25152588Free PMC Article
Small bowel endoscopy and Peutz-Jeghers syndrome.
Korsse SE, Dewint P, Kuipers EJ, van Leerdam ME
Best Pract Res Clin Gastroenterol 2012 Jun;26(3):263-78. doi: 10.1016/j.bpg.2012.03.009. PMID: 22704569

Diagnosis

Updates in the diagnosis and management of non-ampullary small-bowel polyposis.
Lucaciu L, Yano T, Saurin JC
Best Pract Res Clin Gastroenterol 2023 Jun-Aug;64-65:101852. Epub 2023 Jul 22 doi: 10.1016/j.bpg.2023.101852. PMID: 37652652
Clinical Guidelines for Diagnosis and Management of Peutz-Jeghers Syndrome in Children and Adults.
Yamamoto H, Sakamoto H, Kumagai H, Abe T, Ishiguro S, Uchida K, Kawasaki Y, Saida Y, Sano Y, Takeuchi Y, Tajika M, Nakajima T, Banno K, Funasaka Y, Hori S, Yamaguchi T, Yoshida T, Ishikawa H, Iwama T, Okazaki Y, Saito Y, Matsuura N, Mutoh M, Tomita N, Akiyama T, Yamamoto T, Ishida H, Nakayama Y
Digestion 2023;104(5):335-347. Epub 2023 Apr 13 doi: 10.1159/000529799. PMID: 37054692
Peutz-Jeghers syndrome.
Tacheci I, Kopacova M, Bures J
Curr Opin Gastroenterol 2021 May 1;37(3):245-254. doi: 10.1097/MOG.0000000000000718. PMID: 33591027
Management of small bowel polyps: from small to big.
Saurin JC, Benech N, Pioche M
Curr Opin Gastroenterol 2019 May;35(3):250-256. doi: 10.1097/MOG.0000000000000518. PMID: 30844897
Small bowel endoscopy and Peutz-Jeghers syndrome.
Korsse SE, Dewint P, Kuipers EJ, van Leerdam ME
Best Pract Res Clin Gastroenterol 2012 Jun;26(3):263-78. doi: 10.1016/j.bpg.2012.03.009. PMID: 22704569

Therapy

Peutz-Jeghers syndrome without STK11 mutation may correlate with less severe clinical manifestations in Chinese patients.
Jiang LX, Chen YR, Xu ZX, Zhang YH, Zhang Z, Yu PF, Dong ZW, Yang HR, Gu GL
World J Gastroenterol 2023 Jun 7;29(21):3302-3317. doi: 10.3748/wjg.v29.i21.3302. PMID: 37377590Free PMC Article
Safety and efficacy of small bowel polypectomy using a balloon-assisted enteroscope in pediatric patients with Peutz-Jeghers syndrome.
Blanco-Velasco G, Hernández-Mondragón OV, Blancas-Valencia JM, Paz-Flores V, Fuentes-Hernández D, Rodríguez-González P, González-Ortíz B
Rev Gastroenterol Mex (Engl Ed) 2018 Jul-Sep;83(3):234-237. Epub 2018 Feb 12 doi: 10.1016/j.rgmx.2017.07.003. PMID: 29449094
Approach to the endoscopic resection of duodenal lesions.
Gaspar JP, Stelow EB, Wang AY
World J Gastroenterol 2016 Jan 14;22(2):600-17. doi: 10.3748/wjg.v22.i2.600. PMID: 26811610Free PMC Article
Colorectal polyps in childhood.
Thakkar K, Fishman DS, Gilger MA
Curr Opin Pediatr 2012 Oct;24(5):632-7. doi: 10.1097/MOP.0b013e328357419f. PMID: 22890064
Management of small-bowel polyps in Peutz-Jeghers syndrome by using enteroclysis, double-balloon enteroscopy, and videocapsule endoscopy.
Ohmiya N, Nakamura M, Takenaka H, Morishima K, Yamamura T, Ishihara M, Miyahara R, Kawashima H, Itoh A, Hirooka Y, Watanabe O, Ando T, Goto H
Gastrointest Endosc 2010 Dec;72(6):1209-16. doi: 10.1016/j.gie.2010.08.018. PMID: 20970791

Prognosis

Low-level parental mosaicism in an apparent de novo case of Peutz-Jeghers syndrome.
Butel-Simoes GI, Spigelman AD, Scott RJ, Vilain RE
Fam Cancer 2019 Jan;18(1):109-112. doi: 10.1007/s10689-018-0093-3. PMID: 29948449
Small Bowel Neoplasms and Polyps.
Pourmand K, Itzkowitz SH
Curr Gastroenterol Rep 2016 May;18(5):23. doi: 10.1007/s11894-016-0497-x. PMID: 27086004
Update on imaging of Peutz-Jeghers syndrome.
Tomas C, Soyer P, Dohan A, Dray X, Boudiaf M, Hoeffel C
World J Gastroenterol 2014 Aug 21;20(31):10864-75. doi: 10.3748/wjg.v20.i31.10864. PMID: 25152588Free PMC Article
Peutz-Jeghers syndrome: a critical look at colonic Peutz-Jeghers polyps.
Tse JY, Wu S, Shinagare SA, Lauwers GY, Yilmaz O, Wu CL, Deshpande V
Mod Pathol 2013 Sep;26(9):1235-40. Epub 2013 Apr 19 doi: 10.1038/modpathol.2013.44. PMID: 23599156
Vascular, polypoid, and other lesions of the small bowel.
Yano T, Yamamoto H
Best Pract Res Clin Gastroenterol 2009;23(1):61-74. doi: 10.1016/j.bpg.2008.12.001. PMID: 19258187

Clinical prediction guides

Peutz-Jeghers syndrome without STK11 mutation may correlate with less severe clinical manifestations in Chinese patients.
Jiang LX, Chen YR, Xu ZX, Zhang YH, Zhang Z, Yu PF, Dong ZW, Yang HR, Gu GL
World J Gastroenterol 2023 Jun 7;29(21):3302-3317. doi: 10.3748/wjg.v29.i21.3302. PMID: 37377590Free PMC Article
High-Tech Diagnostic Methods and Enteroscopic Treatment of Children with Peutz-Jeghers Syndrome.
Kirakosyan E, Lokhmatov M
Eur J Pediatr Surg 2020 Dec;30(6):529-535. Epub 2019 Nov 26 doi: 10.1055/s-0039-3400286. PMID: 31770782
Clinical and Genetic Study of Children With Peutz-Jeghers Syndrome Identifies a High Frequency of STK11 De Novo Mutation.
Zhao HM, Yang YJ, Duan JQ, Ouyang HJ, Liu L, Yi LC, Xiao ZH, Zheng Y, Peng L, Attard TM, Li DY, You JY
J Pediatr Gastroenterol Nutr 2019 Feb;68(2):199-206. doi: 10.1097/MPG.0000000000002166. PMID: 30334930
Update on imaging of Peutz-Jeghers syndrome.
Tomas C, Soyer P, Dohan A, Dray X, Boudiaf M, Hoeffel C
World J Gastroenterol 2014 Aug 21;20(31):10864-75. doi: 10.3748/wjg.v20.i31.10864. PMID: 25152588Free PMC Article
Peutz-Jeghers syndrome: a critical look at colonic Peutz-Jeghers polyps.
Tse JY, Wu S, Shinagare SA, Lauwers GY, Yilmaz O, Wu CL, Deshpande V
Mod Pathol 2013 Sep;26(9):1235-40. Epub 2013 Apr 19 doi: 10.1038/modpathol.2013.44. PMID: 23599156

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