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[Peutz-Jeghers syndrome and adenocarcinoma. Report of a case]
R R Castro1, C Q Brant, L E Ferreira
1Enfermaria de Gastroenterologia, Hospital São Paulo.
Arquivos De Gastroenterologia
|October 1, 1994
Abstract:
Peutz-Jeghers syndrome is a rare disease, characterized by autosomic inheritance, presenting skin stain and hamartomatous polyps. We report one case of Peutz-Jeghers syndrome and duodenal adenocarcinoma in a young male who presented with liver metastasis and pancreatic invasion. Such association is reported to be presented in 5% of patients with the syndrome. The reasons of such association are unknown, being possible that: 1) there is malignant degeneration of a adenomatous polyp; 2) malignant degeneration of a hamartomatous polyp, or 3) adenomatous portion of a hamartomatous polyp could undergo malignant transformation.