Ján Ušák, Juraj Hromec, Zdena Bartošová, Ivan Vojtech, Vanda Ušáková, Tibor Kružliak
Peutz Jeghers syndrom (PJS) detected by J.L.A. Peutz (1921) and later completely described by H. Jeghers (1944) is an autosomal dominant disorder characterised by intestinal hamartomatous polyps and mucocutaneous melanocytic macules. Genetic backgroud of the disease was explained by Hemminki (1998). Expresion of this rare disease in our population is unknown. In comparison with usual population patients with PJS have 15 times incerased risk of neoplasms. Characteristic pathology of Peutz - Jeghers polyps is extensive smooth muscle arborization throughout the polyp, with the appearance of pseudoinvasion because some of the epithelial cells, usually from benign glands, are surrounded by the smooth muscle.