Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma--therapeutic implications.
نویسندگان
چکیده
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.
منابع مشابه
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ورودعنوان ژورنال:
- Revista espanola de enfermedades digestivas : organo oficial de la Sociedad Espanola de Patologia Digestiva
دوره 101 12 شماره
صفحات -
تاریخ انتشار 2009