Abstract

CIENFUEGOS, J. A. et al. Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma: therapeutic implications. Rev. esp. enferm. dig. [online]. 2009, vol.101, n.12, pp.875-879. ISSN 1130-0108.

The Peutz-Jeghers syndrome (PJS) is an autosomal dominant hamartomatous poliposis describred in 1921. Hemminki in 1997 described the presence of LKB-1 mutation tumor-suppressor gen. The patients with PJS develop a higher cumulative incidence of gastrointestinal, pancreas and extraintestinal tumors, being occasion of a renew interest on hamartomatous polyposis syndromes regarding the clinical care, cancer surveillance treatment and long term follow-up. We report the case of a 38 years old male, diagnosed of PJS who developed a multiple adenocarcinoma in duodenum and yeyunum. Surgically treated and with a long-term free disease survival of 11 years represents the sixth case reported in the spanish literature of PJS associated with a gastrointestinal tumor. A critical review, molecular alterations and the established criteria of tumor screening and surveillance are reviewed.

Keywords : Peutz-Jeghers syndrome; Hamartomatous; Carcinogenesis; Intestinal polyposis; Hereditary cancer.

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