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Intestinal polyposis syndrome

MedGen UID:
577190
•Concept ID:
C0345891
•
Neoplastic Process
Synonyms: Intestinal Polyposis Syndrome; intestinal polyposis syndrome
SNOMED CT: Intestinal polyposis syndrome (254589009)
 
Monarch Initiative: MONDO:0015185
Orphanet: ORPHA104010

Definition

A syndrome associated with the development of multiple polyps throughout the intestine. It includes familial adenomatous polyposis , hamartomatous polyposis syndromes, and other rare polyposis syndromes. [from NCI]

Professional guidelines

PubMed

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
NCCN Guidelines® Insights: Genetic/Familial High-Risk Assessment: Colorectal, Version 1.2021.
Weiss JM, Gupta S, Burke CA, Axell L, Chen LM, Chung DC, Clayback KM, Dallas S, Felder S, Gbolahan O, Giardiello FM, Grady W, Hall MJ, Hampel H, Hodan R, Idos G, Kanth P, Katona B, Lamps L, Llor X, Lynch PM, Markowitz AJ, Pirzadeh-Miller S, Samadder NJ, Shibata D, Swanson BJ, Szymaniak BM, Wiesner GL, Wolf A, Yurgelun MB, Zakhour M, Darlow SD, Dwyer MA, Campbell M
J Natl Compr Canc Netw 2021 Oct 15;19(10):1122-1132. doi: 10.1164/jnccn.2021.0048. PMID: 34666312
ACG clinical guideline: Genetic testing and management of hereditary gastrointestinal cancer syndromes.
Syngal S, Brand RE, Church JM, Giardiello FM, Hampel HL, Burt RW; American College of Gastroenterology
Am J Gastroenterol 2015 Feb;110(2):223-62; quiz 263. Epub 2015 Feb 3 doi: 10.1038/ajg.2014.435. PMID: 25645574Free PMC Article

Recent clinical studies

Etiology

Peutz-Jeghers syndrome: clinicopathology and molecular alterations.
McGarrity TJ, Amos C
Cell Mol Life Sci 2006 Sep;63(18):2135-44. doi: 10.1007/s00018-006-6080-0. PMID: 16952058Free PMC Article
Localization of the Bannayan-Riley-Ruvalcaba syndrome gene to chromosome 10q23.
Zigman AF, Lavine JE, Jones MC, Boland CR, Carethers JM
Gastroenterology 1997 Nov;113(5):1433-7. doi: 10.1053/gast.1997.v113.pm9352843. PMID: 9352843

Diagnosis

Kaposi sarcoma-associated herpesvirus/human herpesvirus 8-associated extracavitary primary effusion lymphoma presenting as multiple lymphomatous polyposis.
Oster C, Stein T, Kitahara S, Alkan S, Huang Q
Hum Pathol 2018 Sep;79:177-183. Epub 2018 Mar 2 doi: 10.1016/j.humpath.2018.02.020. PMID: 29505765
Giant rectal polyp prolapse in an adult patient with the Peutz-Jeghers syndrome.
Cano-Contreras AD, Meixueiro-Daza A, Grube-Pagola P, Remes-Troche JM
BMJ Case Rep 2016 Jul 21;2016 doi: 10.1136/bcr-2016-215629. PMID: 27444139Free PMC Article
A distinct variant of papillary thyroid carcinoma indicating familial adenomatous polyposis (FAP): a case report and brief review.
Liyanapathirana N, Seneviratne SA, Samarasekera DN
BMC Res Notes 2015 Dec 17;8:795. doi: 10.1186/s13104-015-1736-1. PMID: 26681171Free PMC Article
Video capsule endoscopy: what is the future?
Van Gossum A, Ibrahim M
Gastroenterol Clin North Am 2010 Dec;39(4):807-26. doi: 10.1016/j.gtc.2010.08.026. PMID: 21093757

Therapy

Kaposi sarcoma-associated herpesvirus/human herpesvirus 8-associated extracavitary primary effusion lymphoma presenting as multiple lymphomatous polyposis.
Oster C, Stein T, Kitahara S, Alkan S, Huang Q
Hum Pathol 2018 Sep;79:177-183. Epub 2018 Mar 2 doi: 10.1016/j.humpath.2018.02.020. PMID: 29505765
Giant rectal polyp prolapse in an adult patient with the Peutz-Jeghers syndrome.
Cano-Contreras AD, Meixueiro-Daza A, Grube-Pagola P, Remes-Troche JM
BMJ Case Rep 2016 Jul 21;2016 doi: 10.1136/bcr-2016-215629. PMID: 27444139Free PMC Article

Prognosis

Kaposi sarcoma-associated herpesvirus/human herpesvirus 8-associated extracavitary primary effusion lymphoma presenting as multiple lymphomatous polyposis.
Oster C, Stein T, Kitahara S, Alkan S, Huang Q
Hum Pathol 2018 Sep;79:177-183. Epub 2018 Mar 2 doi: 10.1016/j.humpath.2018.02.020. PMID: 29505765
Peutz-Jeghers syndrome: clinicopathology and molecular alterations.
McGarrity TJ, Amos C
Cell Mol Life Sci 2006 Sep;63(18):2135-44. doi: 10.1007/s00018-006-6080-0. PMID: 16952058Free PMC Article

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