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Sporadic Peutz-Jeghers Syndrome with Recurrent Intussusception and Malignant Change in Hamartomatous Polyps

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dc.contributor.authorKumar, Sandeep
dc.contributor.authorGaba, Shivam
dc.contributor.authorTewari, Mallika
dc.contributor.authorJain, Gaurav
dc.date.accessioned2025-01-28T09:47:05Z
dc.date.available2025-01-28T09:47:05Z
dc.date.issued2023
dc.description.abstractPeutz-Jeghers syndrome (PJS) is a rare inherited autosomal dominant disease characterised by mucocutaneous pigmentation and multiple polyps in the gastrointestinal tract. In up to 25% of cases, there is no family history of PJS (sporadic cases). Acute intestinal intussusception due to multiple polyps is the most common complication of PJS. PJS is associated with cancers of the gastrointestinal tract and in other organs. Adenocarcinoma arising in hamartomatous polyps is heavily debated. A 23-year-old gentleman presented with sporadic PJS with recurrent ileal intussusceptions and multifocal well-differentiated adenocarcinomas arising in the background of hamartomatous polyps following hamartoma-dysplasia-carcinoma sequence, which is herein discussed. The hamartoma-dysplasia-carcinoma sequence is one of the pathways of carcinogenesis within hamartomatous polyps in PJS. � 2022, Association of Surgeons of India.
dc.identifier.doihttps://doi.org/10.1007/s12262-022-03528-8
dc.identifier.issn9722068
dc.identifier.urihttps://dl.bhu.ac.in/ir/handle/123456789/22371
dc.language.isoen
dc.publisherSpringer
dc.subjectHamartoma-dysplasia-carcinoma sequence
dc.subjectHamartomatous polyp
dc.subjectPeutz-Jeghers syndrome
dc.subjectRecurrent intussusception
dc.titleSporadic Peutz-Jeghers Syndrome with Recurrent Intussusception and Malignant Change in Hamartomatous Polyps
dc.typeArticle
dspace.entity.typePublication
journal.titleIndian Journal of Surgery
journalvolume.identifier.volume85

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