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

Edward Podczaski, Paul F. Kaminski, Richard C. Pees, Kishor Singapuri, Joel I. Sorosky

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)74-78
Number of pages5
JournalGynecologic Oncology
Volume42
Issue number1
DOIs
StatePublished - Jul 1991

All Science Journal Classification (ASJC) codes

  • Oncology
  • Obstetrics and Gynecology

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