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Case Report
An unusual case of Peutz–Jeghers syndrome with polyposis-associated adenomatous change
1 Senior Resident, Department of General Surgery, Kanachur Institute of Medical Sciences, Mangalore, Karnataka, India
2 Postgraduate, Department of Pathology, AJ Institute of Medical Sciences and Research Centre, Mangalore, Karnataka, India
3 Professor and Head of Department, Department of Pathology, AJ Institute of Medical Sciences and Research Centre, Mangalore, Karnataka, India
Address correspondence to:
Disha Shetty
AJ Institute of Medical Sciences and Research Centre, Mangalore, Karnataka,
India
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Article ID: 101117Z01KS2020
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Shetty KH, Shetty D, Pai MR. An unusual case of Peutz–Jeghers syndrome with polyposis-associated adenomatous change. Int J Case Rep Images 2020;11:101117Z01KS2020.ABSTRACT
Introduction: Peutz–Jeghers syndrome (PJS) is a rare autosomal dominant syndrome associated with mucocutaneous pigmentation and hamartomatous gastrointestinal polyps. It is associated with increased risk of intestinal and extraintestinal malignancies.
Case Report: This is a rare case of PJS with multiple polyps with features of villous adenoma and high grade dysplasia. A 48-year-old female presented with the complaints of bleeding per rectum since five months. Hyperpigmented spots were present on the buccal mucosa. On endoscopy, multiple polyps in distal colon, stomach, duodenum, and periampullary region were noted. Polypectomy was done and sent for histopathological evaluation. Grossly, the largest polyp was in sigmoid colon measuring 2.6 × 1.5 × 1 cm and smallest polyp was in stomach measuring 3 × 2 × 1 mm. On microscopy, polyps from transverse colon showed polypoidal tissue traversed by branching muscularis mucosae forming arborizing pattern. Polyps from sigmoid colon showed similar features with a focus of dysplasia and villous adenoma.
Conclusion: Further the patient was advised to undergo mandatory periodic evaluation on follow-up.
Keywords: Gastrointestinal polyp, Hamartomatous, Peutz–Jeghers syndrome, Polypectomy
SUPPORTING INFORMATION
Author Contributions
Karthik Hariprasad Shetty - Substantial contributions to conception and design, Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Disha Shetty - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Drafting the article, Final approval of the version to be published
Muktha R Pai - Substantial contributions to conception and design, Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2020 Karthik Hariprasad Shetty et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
