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Gynecol Oncol. 1991 Jul;42(1):74-8.

Peutz-Jeghers syndrome with ovarian sex cord tumor with annular tubules and cervical adenoma malignum.

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Department of Obstetrics & Gynecology, M. S. Hershey Medical Center, Pennsylvania State University, Hershey 17033.


A patient with Peutz-Jeghers syndrome, a sex cord tumor with annular tubules, and an initially unrecognized adenoma malignum of the cervix is described. The patient presented with a mucinous adenocarcinoma in the vaginal apex. Review of the hysterectomy slides demonstrated an adenoma malignum of the cervix. In addition to a microscopic sex cord tumor with annular tubules of the right ovary, the left ovary contained mucinous cystadenomas. Adenoma malignum remains a difficult diagnosis and is frequently made only after hysterectomy for a presumed benign indication; pathology frequently demonstrates a deeply invasive, unusually well-differentiated adenocarcinoma of the cervix. Patients with Peutz-Jeghers syndrome need careful clinical and cytologic follow-up to exclude such lesions.

[Indexed for MEDLINE]

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