[Peutz-Jeghers syndrome].
Peutz-Jeghers syndrome is an inherited disorder which usually debuts during childhood. It is characterized by mucocutaneous pigmentation and hamartomatous polyps in the gastrointestinal tract. Numerous reports indicate a high incidence of gastrointestinal and extraintestinal cancer in these patients, their appearance at a young age, as well as its association with ovarian and testicular tumors. An aggressive approach of these patients seems to be necessary. We report the case of two brothers suffering from Peutz-Jeghers syndrome whose father and grandfather died as a consequence of the progression of an intestinal cancer related to the syndrome.
Pérez Rodríguez
M J
MJ
Servicio de Pediatría. Complejo Hospitalario de Ciudad Real. España. Mariaj.p@teleline.es
Anaya Barea
F M
FM
Galiano Fernández
E
E
de Diego Sierra
D
D
Condado Sánchez-Rojas
I
I
D002648:Child; D003937:Diagnosis, Differential; D018450:Disease Progression; D006801:Humans; D008297:Male; D008548:Melanosis / Q000175:diagnosis; D010580:Peutz-Jeghers Syndrome / Q000175:diagnosis*; D012720:Severity of Illness Index