Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma: therapeutic implications

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.

Bibliographic Details

Main Authors:	Cienfuegos,J. A., Baixauli,J., Zozaya,G., Bueno,A., Arredondo,J., Regueira,F. M., Angós,R., Hernández-Lizoáin,J. L., Idoate,M. A.
Format:	Digital revista
Language:	English
Published:	Sociedad Española de Patología Digestiva 2009
Online Access:	http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082009001200009
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