Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma: therapeutic implications
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چکیده
منابع مشابه
Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma--therapeutic implications.
The Peutz-Jeghers syndrome (PJS) is an autosomal dominant hamartomatous poliposis describred in 1921. Hemminki in 1997 described the presence of LKB-1 mutation tumor-suppressor gen.The patients with PJS develop a higher cumulative incidence of gastrointestinal, pancreas and extraintestinal tumors, being occasion of a renew interest on hamartomatous polyposis syndromes regarding the clinical car...
متن کاملPeutz-Jeghers syndrome and metastasising colonic adenocarcinoma.
A case of metastasising colonic carcinoma associated with Peutz-Jeghers syndrome in a 39 year old man is described. The caecal adenocarcinoma had metastasised widely to regional lymph nodes and was associated with several other colonic Peutz-Jeghers polyps, showing no evidence of dysplasia or malignancy. It was not possible to determine whether the carcinoma had arisen from a Peutz-Jeghers poly...
متن کاملJejunal carcinoma in a patient with Peutz-Jeghers syndrome.
P eutz–Jegher syndrome (PJS) is a rare autosomal dominant disorder characterized by typical pigmented perioral macules, pigmented spots in the buccal mucosa and digits along with hamartomatous polyps in the gastrointestinal tract. The characteristic clinical course includes recurrent episodes of polyp-induced bowel obstruction, abdominal pain and gastrointestinal bleeding. In addition to polypo...
متن کاملPeutz-Jeghers syndrome
Introduction Peutz-Jeghers syndrome (PJS) is characterized by: (i) autosomal dominant inheritance; (ii) cutaneous pigmentation; (iii) gastro-intestinal polyposis. In all, more than 300 cases have been described with a world-wide distribution and no racial predilection. In 1921 Peutz described 7 cases of multiple intestinal polyps associated with melanin spots on the lips, buccal mucosa and digi...
متن کامل[Peutz-Jeghers syndrome].
Peutz-Jeghers syndrome is an inherited disorder which usually debuts during childhood. It is characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract. Numerous reports indicate a high incidence of gastrointestinal and extraintestinal cancer in these patients, their appearance at a young age, as well as its association with ovarian and testicular tumors....
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ژورنال
عنوان ژورنال: Revista Española de Enfermedades Digestivas
سال: 2009
ISSN: 1130-0108
DOI: 10.4321/s1130-01082009001200009