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Colonic adenocarcinoma
and bilateral malignant ovarian sex cord tumor with annular tubules in
Peutz-Jeghers syndrome.
Pathologica. 2004 Jun;96(3): 117-20.
Peutz-Jeghers
syndrome is characterized by multiple polyps throughout the
gastrointestinal tract in association with mucocutaneous pigmentation.
Although Peutz-Jeghers syndrome polyps are hamartomas, frequent
association of this syndrome with both gastrointestinal and
non-gastrointestinal tumours had led to reassessment of the cancer
risk in this hereditary disorder. The most common gynaecological
tumors in this syndrome are adenoma malignum of the uterine cervix and
ovarian sex cord tumor, particularly sex cord tumor with annular
tubules. The question of malignant change in a polyp or of the
association of gastro intestinal carcinomas still discuss. The authors
report a case of Peutz-Jeghers syndrome in a young patient who
developed a colonic adenocarcinoma in a hamartomatous polyp together
with an incidentally discovered bilateral malignant sex cord tumours.
We discuss its association with certain benign and malignant tumors
and the risk of rare complications of these hamartomatous polyps.
Although malignant tumors are increasingly reported in association
with the Peutz-Jeghers syndrome, to our knowledge, there have been no
previous reports of such an association in the literature.
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