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Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma-therapeutic implications
Authors: Cienfuegos, J.A. (Javier A.)
Baixauli, J. (Jorge)
Zozaya, G. (Gabriel)
Bueno, A. (Álvaro)
Arredondo, J. (Jorge)
Martinez-Regueira, F. (Fernando)
Angos, R. (Ramón)
Hernandez-Lizoain, J.L. (Jose Luis)
Idoate, M.A. (Miguel Ángel)
Keywords: Peutz-Jeghers syndrome
Intestinal polyposis
Hereditary cancer
Issue Date: 2009
Publisher: Arán Ediciones
Publisher version:
ISSN: 1130-0108
Citation: Cienfuegos JA, Baixauli J, Zozaya G, Bueno A, Arredondo J, Regueira FM, et al. Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma--therapeutic implications. Rev Esp Enferm Dig 2009 Dec;101(12):875-879.
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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