title: [Patient with Peutz-Jeghers syndrome and liver metastases from an unknown primary tumor].
abstract: In a 35-year old man with deep venous thrombosis liver metastases of an adenocarcinoma were observed. The primary tumour was not found. The patient had pigmentations on the lips and hamartomatous polyposis of the intestine. These findings indicate Peutz-Jeghers syndrome. In this syndrome there is an increased risk of, notably, gastrointestinal malignancy at an early age. Recent investigations have shown that Peutz-Jeghers syndrome is associated with a mutation in the STK11 gene on chromosome 19.
authors.affiliation: Afd. Inwendige Geneeskunde, Academisch Ziekenhuis Vrije Universiteit, Amsterdam.
authors.forename: G
authors.initials: G
authors.lastname: van der Werf
authors.forename: F H
authors.initials: FH
authors.lastname: Menko
authors.forename: J J
authors.initials: JJ
authors.lastname: Gille
authors.forename: P J
authors.initials: PJ
authors.lastname: van Diest
authors.forename: C J
authors.initials: CJ
authors.lastname: van Groeningen
mesh_terms: D000230:Adenocarcinoma / Q000209:etiology / Q000556:secondary; D000328:Adult; D020022:Genetic Predisposition to Disease; D006222:Hamartoma / Q000209:etiology / Q000473:pathology; D006801:Humans; D007414:Intestinal Neoplasms / Q000209:etiology / Q000473:pathology; D008113:Liver Neoplasms / Q000209:etiology / Q000556:secondary; D008297:Male; D009382:Neoplasms, Unknown Primary / Q000235:genetics; D010580:Peutz-Jeghers Syndrome / Q000150:complications* / Q000235:genetics / Q000473:pathology*; D013924:Thrombophlebitis / Q000209:etiology