Polyposis colon - intestinal polyposis syndromes

Polyposis colon - intestinal polyposis syndromes

    * Introduction
    * Signs and symptoms
    * Diagnosis
    * Treatment
Intestinal polyposis is a serious hereditary disease that occurs in puberty, characterized by the appearance of multiple polyps in the intestinal mucosa. The most common intestinal polyposis is manifested by diffuse abdominal pain, intestinal bleeding (bloody stools) and changes in bowel habits. Have been described several hereditary syndromes that have as common factor intestinal polyps.
Gardner first published an article on familial polyposis, colon polyps in describing a Utah family whose members had died from colon cancer in September over three generations, at a mean age of 34 years. Some genetic abnormalities present with gastrointestinal polyps. Individuals with Gardner syndrome develop adenomatous polyps throughout the gastrointestinal tract accompanied by extracolonice events, including periampulare adenoma, papillary thyroid carcinoma, hepatoblastom, osteomas of the mandible and skull, epidermal cysts and dermoid tumors.
Individuals with Turcot syndrome, a rare recessive autosomal disorder, shows brain tumors (glioblastoma multiforme, medulloblastoma) and which often become malignant colonic adenomas in patients younger than 30 years.
Initially described the Peutz-Jeghers syndrome Peutz Jeghers in 1921 followed in 1941. Malignant degeneration is characterized by gastrointestinal polyps and extraintestinal neoplasms development, including the pancreas, and the gynecologic testiculii. The development of gynecomastia usually precedes the development of testicular or gynecologic neoplasms. In Peutz-Jeghers syndrome polyps can occur anywhere in the digestive tract, most commonly in the jejunum, accompanied by melanin spots of the lips and fingers.
Bannayan-Riley-Ruvalcaba syndrome known as Bannayan-Zonana syndrome was described by Riley and Smith in 1961, then Bannayana in 1971 and later characterized by Zonana in 1975. This associate of colon polyps and language hamartosi with macrocephaly, lipomas and hemangiomas.
Gorlin and Goltz described Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome. It is characterized by gastric polyps hamartosi, palm spots, short metacarpal, keratocisti odontogenic, intracranial calcifications, skeletal malformations and cancer (basal cell carcinoma, ovarian carcinoma, medulloblastoma).
People Cronkhite-Canada syndrome shows multiple abnormalities in the intestinal polyps actodermice, including skin hyperpigmentation, alopecia and onicoheterotopie.
Cowden syndrome is an autosomal dominant hereditary disorder. People with this syndrome shows at the age of 10-30 years hamartosi hyperplastic polyps in the gastrointestinal tract, including esophagus, the esophageal glycogen acanthosis, hamartoame orocutanate the face, breast neoplasia hamartoame lung, thyroid, colon adenocarcinoma.
Inflammatory polyps are discovered in 1% of children. Multiple inflammatory polyps associated with painless rectal bleeding, rectal prolapse and no seat colony characterize juvenile polyposis. Malignant potential is much higher compared to Gardner's syndrome.