peutz-jeghers syndrome report of one case associated with gastrointestin carcinoma
نویسندگان
چکیده
the peutz-jeghers syndrome is characterized by an association of gastrointestinal polyposis with rnelain spots on oral mucosa, lips, and skin. this symdrorne is inherited as a simple mendelian autosomal dominant trait. intussusception is by far the most common complication. although these polyps are widely regarded as hamartomas and rarely undergo malignant change, they have been reported to be associated with carcinoma of gastrointestin and ovary. in the case reported here, the cancer developed from hamartomatous polyps.
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 کاملPeutz - Jeghers syndrome associated with gastrointestinal carcinoma
Patients with the Peutz-Jeghers syndrome carry a slight, though definite, increased risk of gastrointestinal carcinoma.The malignant potentiality of Peutz-Jeghers hamartomatous polyps, generally considered benign, is supported by this report. Two cases of metastasising gastrointestinal carcinoma associated with the Peutz-Jeghers syndrome are described in a 56 year old female and her 29 year old...
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 کاملPeutz-jeghers Syndrome with Metastasizing Duodenal Carcinoma.
EDITORIAL SYNOPSIS Since interest was first aroused in the Peutz-Jeghers syndrome there have been conflicting views as to the frequency with which malignant change occurs in the polyps found in the small intestine, and this has been due to the difficulty of interpreting the histological structure of the polypi. Many now feel that in this syndrome these lesions are hamartomas and that the appare...
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...
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عنوان ژورنال:
acta medica iranicaجلد ۲۴، شماره ۳-۴، صفحات ۱۱۵-۱۲۹
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