[1] While the majority of the polyps found in juvenile polyposis syndrome are non-neoplastic, hamartomatous, self-limiting and benign, there is an increased risk of adenocarcinoma.

The World Health Organization criteria for diagnosis of juvenile polyposis syndrome are one of either: Age of onset is variable.

The term 'juvenile' in the title of juvenile polyposis syndrome refers to the histological type of the polyps rather than the age of onset.

[1] Juvenile polyposis syndrome can occur sporadically in families or be inherited in an autosomal dominant manner.

The cumulative lifetime risk of colorectal cancer is 39% in patients with juvenile polyposis syndrome.