Hamartomatous Polyposis

Polyps in both PJS and familial JPS are hamartoma-tous, but the major tissue components are different. The Peutz-Jeghers polyp is composed of intestinal crypts and villi and smooth-muscle bundles in a disorganised fashion. Inflammatory cells are absent or scanty, and glandular structures are not excessively dilated. The juvenile polyp is composed of dilated intestinal glands and abundant connective tissue of the lamina propria. Smooth-muscle elements are usually absent; lymphoid cells are common. These features closely resemble those of inflammatory retention polyps in children, also known as juvenile polyps, as well as inflammatory polyps of Cronkhite-Canada syndrome, which occur among the elderly.

PJS is characterized by the presence of Peutz-Jeghers polyps throughout the entire GIT and melanin spots on the lip (96%), buccal mucosa (83%), face (36%) and extremities (32%) [8].The small bowel is the favoured site of polyposis and the number of polyps is small. Other associated abnormalities include polyps in the urinary bladder and nasal cavity, bone deformities, congenital heart disease and retarded development. The symptoms of PJS develop before the age of 20 in two-thirds of cases, with an average age at time of diagnosis of 22 years [3]. Symptoms are noted at a younger age than those associated with FAP. Peutz-Jeghers polyps are generally not prone to malignant change. However, carcinoma of the GIT has been reported in about 3% of cases, most commonly in the proximal small intestine [9]. In many reports, the relationship between polyp and carcinoma is unclear. When the origin of malignancy was carefully studied, the carcinoma was usually found to arise in the adenomatous or dys-plastic epithelium in the polyp [10]. Malignancy can also occur outside of the GIT. The condition is linked to 19p chromosomes in at least some families.

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