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

Gynecol Oncol. 1991 Jul;42(1):74-8. doi: 10.1016/0090-8258(91)90234-v.


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.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma / complications*
  • Adenocarcinoma / pathology
  • Adenocarcinoma / radiotherapy
  • Adolescent
  • Female
  • Humans
  • Hysterectomy
  • Neoplasm Recurrence, Local
  • Neoplasms, Multiple Primary*
  • Ovarian Neoplasms / complications*
  • Ovarian Neoplasms / pathology
  • Ovarian Neoplasms / surgery
  • Peutz-Jeghers Syndrome / complications*
  • Peutz-Jeghers Syndrome / surgery
  • Uterine Cervical Neoplasms / complications*
  • Uterine Cervical Neoplasms / pathology
  • Uterine Cervical Neoplasms / radiotherapy