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Juvenile Polyposis may or may not be familial. Familial Juvenile Polyposis Juvenile polyposis syndrome (JPS) is a rare autosomal dominant hereditary disorder characterized by multiple distinct juvenile polyps in the gastrointestinal tract. Juvenile polyps are hamartomatous lesions in the gastrointestinal (GI) tract with a distinct histological appearance of normal epithlium with cystic glands embedded in hyperplastic stroma and inflammatory infiltrate. Juvenile polyps are typically benign, but in individuals with juvenile polyposis syndrome (JPS). Juvenile Polyposis can cause polyps in the large intestine, small intestine and stomach. Usually, colorectal polyps are the ones that cause symptoms, such as bleeding, diarrhea, abdominal cramps and anemia. Juvenile Polyp of the Gastrointestinal Tract Definition Gastrointestinal polyp with prominent cystically dilated glands and inflammatory stroma. Clinical Features and Diagnosis Juvenile polyposis syndrome (JPS) is a rare autosomal-dominant disorder associated with an increased risk of colorectal cancer (68% by age 60 years) and upper gastrointestinal tract cancer (10%). Juvenile polyps are histologically distinct and consist of dilated cysts filled with mucin and lined by stromal cells. Cancers appear to arise from adenomatous components present in some juvenile polyps. The number of polyps required to establish the diagnosis remains controversial, but most. agree that patients with between three and five juvenile polyps or any number of juvenile polyps found in a patient with a family history of JPS should undergo evaluation. Juvenile polyps can also occur in Cowden syndrome (facial trichilemmomas, nontoxic multinodular goiter, thyroid and breast cancer), and Bannayan-Riley-Ruvalcaba syndrome (macrocephaly, hyperpigmentation of the genitalia, slowed psychomotor development).