Year 2009 / Volume 101 / Number 12
Notas
Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma - therapeutic implications

pp. 875-879

Cienfuegos, J. A. / Baixauli, J. / Zozaya, G. / Bueno, A. / Arredondo, J. / Regueira, F. M. / Angós, R. / Hernández Lizoáin, J. L. / Idoate, M. A.

Abstract
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.
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Cienfuegos, J. A. / Baixauli, J. / Zozaya, G. / Bueno, A. / Arredondo, J. / Regueira, F. M. / Angós, R. / Hernández Lizoáin, J. L. / Idoate, M. A.. Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma - therapeutic implications. 875-879


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