Peutz-Jeghers sendromu

Tuğba Han Yılmaz; Tevfik Avcı; Varlık Erol; Hüseyin Gülay

doi:10.19161/etd.344215

EN TR

Peutz-Jeghers syndrome

Abstract

Peutz-Jeghers syndrome (PJS) is an autosomally dominant inherited disease which is responsible for mucocutaneous pigmentation and gastrointestinal polyps. Round, oval or irregular patches of brown pigmentation 1-5 mm in diameter, distributed over the oral mucosa, gums, hard palate and lips are observed. Most of the polyps reside in the jejunum, it may also ocur in ileum, stomach, duodenum and/or colon. There is a higher risk of intestinal and extraintestinal cancers in those patients. Herein we present a 44-year-old male patient having intermittent episodes of bloating and abdominal pain without a particular localization, as well as mild iron deficiency anemia for ten years. Physical examination revealed pigmented lesions on oral mucosa and fingertips, palpable mass on abdominal left upper quadrant, and colonoscopy showed multiple hamartomatous polyps. At the operation performed for the mesenteric mass, it was detected that 2 of 8 small intestinal polyps have intramucosal carsinoma and the mesenteric mass was reported as signet ring cell carsinoma. Patients with PJS should be regularly and closely monitored, because of the increased risk of cancer.

Keywords

Peutz-Jeghers syndrome, cancer, follow-up and treatment

Details

Primary Language

Turkish

Subjects

-

Journal Section

-

Authors

Tuğba Han Yılmaz

Tevfik Avcı

Varlık Erol

Hüseyin Gülay

Publication Date

September 1, 2016

Submission Date

September 1, 2016

Acceptance Date

-

Published in Issue

Year 2016 Volume: 55 Number: 3

DOI

https://doi.org/10.19161/etd.344215

IZ

https://izlik.org/JA48XL47MH

Cite

RIS / Bibtex

Vancouver

1.Tuğba Han Yılmaz, Tevfik Avcı, Varlık Erol, Hüseyin Gülay. Peutz-Jeghers sendromu. EJM. 2016 Sep. 1;55(3):152-4. doi:10.19161/etd.344215

Peutz-Jeghers syndrome

Peutz-Jeghers syndrome

Abstract

Keywords