Massive gastric polyposis in Peutz-Jeghers Syndrome – a case report
نویسندگان
چکیده
Peutz-Jeghers Syndrome (PJS) is a rare autosomal dominant disorder equally affecting males and females with an incidence of 1/25,000 – 1/300,000 births. It characterised by the development hamartomatous polyps digestive tract, lifetime risk colorectal cancer other gastrointestinal malignancies, as well non-gastrointestinal cancers such breast ovarian cancers. Intestinal polyposis very common in PJS, however, gastric occur less than 20% patients massive rarely reported. We report case elderly PJS patient concurrent prior intestinal multiple occurrences intussusception. The were present fundus through to gastro-eosophageal junction. Microscopy demonstrated arborising branches smooth muscle covered disorganised mucosa consistent polyps. largest polyp showed architectural complexity nuclear atypia suggestive high-grade dysplasia. An intrapolyp adenocarcinoma had been biopsied from two months but no residual invasion was gastrectomy specimen. important for pathologists be aware association between which dysplasia malignancy can develop.
منابع مشابه
Peutz-Jeghers syndrome, case report.
Peutz-Jeghers syndrome (PJS) is an unusual hamartomatous polyposis of the gastro intestinal (GI) tract, with pigmentation around lips and macules on the buccal mucosa. The case of a 10-year-old girl who presented with intussusception is reported. A polyp was found to be the cause of an invagination. Histologically it was a hamartoma. PJS is a rare syndrome inherited in an autosomal dominant pat...
متن کاملThe Peutz Jeghers Syndrome: A Case Report
Peutz Jeghers syndrome ( PJ S ) is an autosomal dominant disease that combines hamartomatous polyposis ,a periorificial lentiginose and a high risk of associated cancers. We report the observation of a girl 07 years old of personal historyof acute intestinal intussusception occurred a year ago who consults for signs of early puberty and the onset of vaginal bleeding up to 06 months . The child ...
متن کاملPeutz-Jeghers' Syndrome. A case report.
Peutz-Jeghers syndrome (PJS) is an unusual hamartomatous polyposis of the gastro intestinal (GI) tract, with pigmentation around lips and macules on the buccal mucosa. The case of a 10-year-old girl who presented with intussusception is reported. A polyp was found to be the cause of an invagination. Histologically it was a hamartoma. PJS is a rare syndrome inherited in an autosomal dominant pat...
متن کاملCASE REPORT Peutz–Jeghers syndrome: case report and literature review
Periorificial lentiginosis, also knew as Peutz–Jeghers Syndrome (PJS), is an autosomally dominant inherited condition determined by a mutation localized at 19p13.3 responsible for mucocutaneous pigmentation and gastrointestinal polyps. Skinand mucosal pigmentation may be present at birth but usually occur in early childhood, and occasionally may develop later. Round, oval or irregular patches o...
متن کاملMelaena with Peutz-Jeghers syndrome: a case report
INTRODUCTION Peutz-Jeghers syndrome (PJS) is a rare familial disorder characterised by mucocutaneous pigmentation, gastrointestinal and extragastrointestinal hamartomatous polyps and an increased risk of malignancy. Peutz-Jeghers polyps in the bowel may result in intussusception. This complication usually manifests with abdominal pain and signs of intestinal obstruction. CASE PRESENTATION We ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Pathology
سال: 2023
ISSN: ['1465-3931', '0031-3025']
DOI: https://doi.org/10.1016/j.pathol.2022.12.217