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.
Publication types
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Case Reports
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Comparative Study
MeSH terms
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Adenocarcinoma* / diagnostic imaging
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Adenocarcinoma* / mortality
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Adenocarcinoma* / pathology
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Adenocarcinoma* / surgery
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Adolescent
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Adult
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Child
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Child, Preschool
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Disease-Free Survival
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Duodenal Neoplasms* / diagnostic imaging
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Duodenal Neoplasms* / mortality
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Duodenal Neoplasms* / pathology
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Duodenal Neoplasms* / surgery
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Female
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Follow-Up Studies
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Humans
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Infant
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Infant, Newborn
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Jejunal Neoplasms* / diagnostic imaging
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Jejunal Neoplasms* / mortality
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Jejunal Neoplasms* / pathology
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Jejunal Neoplasms* / surgery
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Male
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Neoplasm Invasiveness / pathology
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Neoplasms, Multiple Primary* / diagnostic imaging
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Neoplasms, Multiple Primary* / mortality
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Neoplasms, Multiple Primary* / pathology
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Neoplasms, Multiple Primary* / surgery
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Pancreaticoduodenectomy
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Peutz-Jeghers Syndrome / complications*
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Radiography, Abdominal
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Time Factors
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Tomography, X-Ray Computed
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Treatment Outcome