Peutz-Jeghers syndrome and duodeno-jejunal adenocarcinoma--therapeutic implications.
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.
Cienfuegos
J A
JA
Departments of General Surgery, Digestive Diseases, Clínica Universidad de Navarra, Pamplona,Spain. fjacien@unav.es
Baixauli
J
J
Zozaya
G
G
Bueno
A
A
Arredondo
J
J
Regueira
F M
FM
Angós
R
R
Hernández-Lizoáin
J L
JL
Idoate
M A
MA
D000230:Adenocarcinoma / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004379:Duodenal Neoplasms / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007580:Jejunal Neoplasms / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology / Q000601:surgery; D008297:Male; D009361:Neoplasm Invasiveness / Q000473:pathology; D009378:Neoplasms, Multiple Primary / Q000000981:diagnostic imaging / Q000401:mortality / Q000473:pathology / Q000601:surgery; D016577:Pancreaticoduodenectomy; D010580:Peutz-Jeghers Syndrome / Q000150:complications*; D011860:Radiography, Abdominal; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome