open access: Frequency and Spectrum of Cancers in the Peutz-Jeghers Syndrome (2006)

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 ".....Peutz-Jeghers syndrome (MIM:175200; ref. 1) is an autosomal dominantly inherited syndrome characterized by mucocutaneous pigmentation and gastrointestinal polyposis (2). In addition to an elevated risk of gastrointestinal cancers, an increased risk of cancers at other sites, such as breast, ovary, uterus, cervix, lung, and testis, has been described (3–7). Rare tumors have also been attributed to Peutz-Jeghers syndrome, including testicular sex cord and Sertoli cell tumors, leading to sexual precocity and gynecomastia (8–10), ovarian sex cord tumors with annular tubules, and adenoma malignum of the cervix (1, 2, 11, 12)"