The peutz-Jeghers syndrome, a condition characterized by harmatomatous polyps of the gastrointestinal tract and mucocutaneous pigmentations, appears to be transmitted in an autosomal dominant fashion, but de nove cases have been reported without
any
suggestive family history. The mucocutancous pigmentations are usually found on and around the lips and buccal mucosa, fingers and toes. The harmatomatous polyps which are predominantly located in the small bowel, particularly the jejunum but
also
can
occur in the stomach, colon and rectum are not generally regarded to be premalignant. But in patinents with this syndrome, increased rates of both intestinal and extraintestinal malignancies has been reported.
The authors here present a case of clinical experience of a 33-year old male patients with Peutz-jeghers syndrome who had an advanced adenocarcinoma at the rectum near the rectosigmoid junction and metastatic hepatic cancer with review of
literatures.
|