Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma-therapeutic implications
| Autor: | Álvarez-Cienfuegos, J. (Javier), Baixauli, J. (Jorge), Zozaya, G. (Gabriel), Bueno, A. (Álvaro), Blasco-Blanco, M. (Manuel), Martinez-Regueira, F. (Fernando), Angós, R. (Ramón), Hernandez-Lizoain, J.L. (Jose Luis), Idoate, M.A. (Miguel Ángel) |
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| Jazyk: | Spanish; Castilian |
| Rok vydání: | 2009 |
| Předmět: | |
| Zdroj: | Dadun. Depósito Académico Digital de la Universidad de Navarra instname |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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